30 types blood and body fluid system disease treatment investigation finding differential diagnosis contraindications ICU and OPD and blood transfusion and type of anemia diagnosis summary
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Here is a comprehensive clinical reference covering 30 Blood & Body Fluid System Diseases — including treatment, investigations, findings, differential diagnoses, contraindications, ICU/OPD management, blood transfusion indications, and anemia classification.
🩸 30 BLOOD & BODY FLUID SYSTEM DISEASES — CLINICAL REFERENCE
PART A: ANEMIAS (Types 1–9)
1. IRON DEFICIENCY ANEMIA (IDA)
| Feature | Detail |
|---|---|
| Type | Microcytic, hypochromic anemia |
| Cause | Inadequate intake, chronic blood loss (GI, menstrual), malabsorption |
| Findings | Pallor, koilonychia, angular stomatitis, glossitis, Plummer-Vinson syndrome (dysphagia) |
| Investigations | CBC: ↓Hb, ↓MCV, ↓MCH; Serum ferritin ↓ (<12 ng/mL); Serum iron ↓; TIBC ↑; Peripheral smear: pencil cells, target cells; Reticulocyte count ↓ |
| Diagnosis Summary | Serum ferritin <12 ng/mL is diagnostic; ↓iron + ↑TIBC confirms |
| Differential Diagnosis | Thalassemia trait, anemia of chronic disease, sideroblastic anemia, lead poisoning |
| Treatment | Oral ferrous sulfate 325 mg TDS × 3–6 months; IV iron (ferric carboxymaltose) if malabsorption or intolerant; treat underlying cause |
| Contraindications | Iron therapy contraindicated in hemochromatosis, hemolytic anemia without iron deficiency |
| Blood Transfusion | Only if Hb <7 g/dL with symptomatic hemodynamic compromise; packed RBCs preferred |
| OPD | Dietary counseling, oral iron supplementation, follow-up CBC at 4 weeks |
| ICU | Transfuse if Hb <7 (or <8 in cardiac patients); IV iron infusion; monitor for anaphylaxis |
2. VITAMIN B12 DEFICIENCY ANEMIA (Megaloblastic)
| Feature | Detail |
|---|---|
| Type | Macrocytic, megaloblastic anemia |
| Cause | Pernicious anemia (anti-IF antibodies), gastrectomy, strict veganism, Crohn's disease, fish tapeworm |
| Findings | Pallor, glossitis, subacute combined degeneration of spinal cord (SACD), hypersegmented neutrophils, lemon-yellow jaundice |
| Investigations | CBC: ↑MCV >100 fL; Peripheral smear: macro-ovalocytes, hypersegmented neutrophils; Serum B12 <200 pg/mL; LDH ↑; Indirect bilirubin ↑; Schilling test (historical); Anti-intrinsic factor antibodies |
| Diagnosis Summary | Serum B12 <200 pg/mL + megaloblastic marrow + neurological features |
| Differential Diagnosis | Folate deficiency, hypothyroidism, liver disease, myelodysplastic syndrome |
| Treatment | Cyanocobalamin IM 1000 mcg daily × 7 days → weekly × 4 → monthly (lifelong in pernicious anemia); oral B12 1000–2000 mcg/day if dietary |
| Contraindications | Avoid folate replacement alone if B12 deficiency present (worsens SACD) |
| Blood Transfusion | Rarely needed; if severely symptomatic Hb <7 g/dL; transfuse slowly (risk of fluid overload due to expanded plasma volume) |
| OPD | Lifelong B12 injections in pernicious anemia; dietary supplementation; neurological monitoring |
| ICU | Severe pancytopenia → supportive care; monitor for cardiac arrhythmias |
3. FOLATE DEFICIENCY ANEMIA
| Feature | Detail |
|---|---|
| Type | Macrocytic, megaloblastic anemia |
| Cause | Poor diet (alcohol, elderly), pregnancy, hemolytic anemia (↑demand), methotrexate, phenytoin, trimethoprim |
| Findings | Pallor, glossitis, no neurological features (unlike B12 deficiency) |
| Investigations | CBC: ↑MCV; Serum folate <3 ng/mL; RBC folate <140 ng/mL (more reliable); Peripheral smear: hypersegmented neutrophils; Normal B12 level |
| Diagnosis Summary | ↓Serum/RBC folate + megaloblastic smear + no neurological signs |
| Differential Diagnosis | B12 deficiency, drug-induced megaloblastosis, MDS |
| Treatment | Folic acid 5 mg/day orally × 4 months; prophylaxis: 400 mcg/day in pregnancy; stop offending drugs |
| Contraindications | Rule out B12 deficiency FIRST before folate therapy (to prevent unmasking SACD) |
| Blood Transfusion | Only if Hb <7 with symptoms |
| OPD | Dietary education; supplementation in pregnancy and hemolytic conditions |
| ICU | Supportive; manage severe pancytopenia |
4. APLASTIC ANEMIA
| Feature | Detail |
|---|---|
| Type | Pancytopenia due to bone marrow failure |
| Cause | Idiopathic (autoimmune, most common), drugs (chloramphenicol, NSAIDs, chemotherapy), infections (hepatitis, EBV, CMV, parvovirus B19), radiation, PNH |
| Findings | Pallor, purpura/petechiae, bleeding, infections (neutropenic fever), hypocellular bone marrow |
| Investigations | CBC: pancytopenia; Reticulocyte count ↓; Bone marrow biopsy: hypocellular marrow (<25% cellularity) — GOLD STANDARD; LFTs; viral serology; Ham's test (for PNH); Flow cytometry (CD55/CD59) |
| Diagnosis Summary | Severe aplastic anemia: ≥2 of — neutrophils <0.5×10⁹/L, platelets <20×10⁹/L, reticulocytes <20×10⁹/L + hypocellular marrow |
| Differential Diagnosis | MDS, PNH, leukemia, B12/folate deficiency, hypersplenism |
| Treatment | Young <40 years + HLA-matched sibling: allogeneic HSCT (curative); Others: anti-thymocyte globulin (ATG) + cyclosporine + eltrombopag; G-CSF (filgrastim) for neutropenia; Androgens (danazol) in elderly |
| Contraindications | Avoid live vaccines; avoid nephrotoxins with cyclosporine; HSCT contraindicated in uncontrolled infection |
| Blood Transfusion | Transfuse RBCs if Hb <7; platelets if <10×10⁹/L or active bleeding; use CMV-negative, irradiated products |
| OPD | Cyclosporine monitoring (drug levels, renal function); infection prophylaxis (fluconazole, acyclovir) |
| ICU | Neutropenic sepsis protocol; broad-spectrum antibiotics; reverse isolation; transfusion support |
5. HEMOLYTIC ANEMIA
| Feature | Detail |
|---|---|
| Type | Normocytic anemia with ↑RBC destruction |
| Causes | Intrinsic: G6PD deficiency, hereditary spherocytosis, sickle cell, thalassemia, PNH; Extrinsic: AIHA, microangiopathic (TTP/HUS), drugs, infections, hypersplenism |
| Findings | Pallor, jaundice (indirect bilirubinemia), splenomegaly, dark urine (hemoglobinuria in intravascular), gallstones |
| Investigations | CBC: ↓Hb, ↑MCV (reticulocytes); Reticulocyte count ↑↑; Peripheral smear: spherocytes, schistocytes, sickle cells; DAT (Coombs test) — positive in AIHA; LDH ↑; Indirect bilirubin ↑; Haptoglobin ↓ (intravascular); Urine hemosiderin |
| Diagnosis Summary | ↑Reticulocytes + ↑LDH + ↓haptoglobin + ↑indirect bilirubin |
| Differential Diagnosis | Hemorrhagic anemia, ineffective erythropoiesis, liver disease |
| Treatment | AIHA: Prednisolone 1 mg/kg/day → taper; IVIG; rituximab; splenectomy; G6PD: avoid triggers; Hereditary spherocytosis: splenectomy; PNH: eculizumab |
| Contraindications | Avoid splenectomy in children <5 yrs; avoid oxidant drugs in G6PD deficiency (dapsone, primaquine, nitrofurantoin) |
| Blood Transfusion | Use washed/leukoreduced RBCs; cross-match carefully (AIHA has panagglutinins); transfuse if Hb <7 or symptomatic |
| OPD | Monitor Hb and reticulocytes; folic acid supplementation; vaccination pre-splenectomy (pneumococcal, meningococcal, Hib) |
| ICU | Hemolytic crisis: IV fluids, bicarbonate (renal protection), RBC transfusion, treat precipitating cause; TTP: emergency plasmapheresis |
6. SICKLE CELL DISEASE (SCD)
| Feature | Detail |
|---|---|
| Type | Inherited hemolytic anemia; HbSS genotype |
| Findings | Vaso-occlusive crisis (severe pain), dactylitis, acute chest syndrome, stroke, splenic sequestration, priapism, avascular necrosis, chronic pain, susceptibility to encapsulated organisms |
| Investigations | Peripheral smear: sickle cells, target cells; Hb electrophoresis (HbS >90%); Sickling test; CBC: Hb 6–9 g/dL; Reticulocyte count ↑; LDH ↑; Renal/liver function; MRI brain (stroke); Echo (pulmonary hypertension) |
| Diagnosis Summary | Hb electrophoresis showing HbSS + peripheral smear findings |
| Differential Diagnosis | HbSC disease, sickle-beta thalassemia, G6PD deficiency |
| Treatment | Crisis: IV fluids, strong analgesia (morphine/PCA), oxygen; Chronic: Hydroxyurea (↑HbF, reduces crisis frequency); L-glutamine; Crizanlizumab; Voxelotor; Folic acid 5 mg/day; Curative: HSCT, gene therapy |
| Contraindications | Hydroxyurea: avoid in pregnancy; avoid dehydration, hypoxia, cold exposure |
| Blood Transfusion | Simple or exchange transfusion in stroke, acute chest, splenic sequestration, pre-surgery; target HbS <30% for exchange; use phenotypically matched, leukoreduced blood |
| OPD | Hydroxyurea monitoring (CBC), vaccination (pneumococcal, meningococcal, flu), penicillin prophylaxis (children), transcranial Doppler screening |
| ICU | Acute chest syndrome: exchange transfusion + bronchodilators + antibiotics; stroke: emergency exchange transfusion; multiorgan failure: ICU support |
7. THALASSEMIA
| Feature | Detail |
|---|---|
| Type | α-Thalassemia (α-globin gene deletion) and β-Thalassemia (β-globin gene mutation) |
| Classification | β-Thal major (Cooley's anemia), β-Thal intermedia, β-Thal minor (trait); Hb H disease, Hb Barts (α-Thal major, hydrops fetalis) |
| Findings | Pallor, jaundice, splenomegaly, hepatomegaly, "chipmunk facies" (frontal bossing, malocclusion), iron overload, growth retardation |
| Investigations | CBC: ↓Hb, ↓MCV, ↓MCH; Peripheral smear: target cells, nucleated RBCs, teardrops; Hb electrophoresis: ↑HbA2 (>3.5%) in β-Thal minor; ↑HbF; DNA analysis (α-Thal); Serum ferritin (iron overload); LFTs; Echo (cardiac siderosis) |
| Diagnosis Summary | Hb electrophoresis + genetic testing + family history |
| Differential Diagnosis | IDA, HbE disease, sideroblastic anemia, hemoglobin C disease |
| Treatment | β-Thal major: Regular transfusions every 2–4 weeks (target Hb >9–10 g/dL); Iron chelation — deferoxamine (SC infusion) or deferasirox (oral) or deferiprone; Folic acid; Curative: HSCT, gene therapy (betibeglogene); Splenectomy if hypersplenism |
| Contraindications | Avoid iron therapy unless proven deficiency; defer chelation if ferritin <1000 ng/mL |
| Blood Transfusion | Regular leukoreduced, phenotypically matched PRBCs; pre-transfusion Hb <9 g/dL triggers transfusion |
| OPD | Monthly CBC; ferritin monitoring; endocrine surveillance (growth, thyroid, glucose); bone density; audiogram (deferoxamine toxicity) |
| ICU | Cardiac failure from iron overload: continuous IV deferoxamine; arrhythmia management; transfusion for severe anemia |
8. ANEMIA OF CHRONIC DISEASE (ACD)
| Feature | Detail |
|---|---|
| Type | Normocytic/microcytic anemia; functional iron deficiency |
| Cause | Chronic infection, autoimmune disease (RA, SLE), malignancy, CKD, inflammatory bowel disease |
| Mechanism | ↑Hepcidin → ↓iron absorption + ↓iron release from macrophages + ↓EPO response |
| Investigations | CBC: Hb 8–10 g/dL, normal/↓MCV; Serum iron ↓; TIBC ↓ (unlike IDA); Ferritin ↑ (acute phase reactant); Transferrin saturation ↓; Soluble transferrin receptor (sTfR): normal |
| Diagnosis Summary | ↓Fe, ↓TIBC, ↑ferritin in context of chronic illness — distinguishes from IDA |
| Differential Diagnosis | IDA, thalassemia trait, sideroblastic anemia |
| Treatment | Treat underlying disease; ESAs (erythropoietin, darbepoetin) in CKD/chemotherapy; IV iron if concurrent iron deficiency; RBC transfusion if symptomatic |
| Contraindications | ESAs contraindicated if Hb >12 g/dL; avoid ESAs in active malignancy (risk of thrombosis/tumor progression) |
| OPD | Monitor Hb, ferritin, transferrin saturation; treat underlying inflammation |
| ICU | Transfuse if Hb <7 (restrictive strategy); monitor for fluid overload in CKD |
9. SIDEROBLASTIC ANEMIA
| Feature | Detail |
|---|---|
| Type | Microcytic/normocytic; ring sideroblasts in bone marrow |
| Cause | Hereditary (ALAS2 mutation, X-linked), acquired (MDS, alcohol, lead, isoniazid, chloramphenicol, copper deficiency) |
| Findings | Pallor, hepatosplenomegaly, iron overload features |
| Investigations | CBC: ↓Hb, dimorphic RBCs; Serum iron ↑; TIBC ↓; Ferritin ↑; Bone marrow: ring sideroblasts (Prussian blue stain) — ≥15% of erythroblasts; Lead levels; ALA (if lead toxicity) |
| Diagnosis Summary | Ring sideroblasts on bone marrow Prussian blue staining |
| Differential Diagnosis | IDA, thalassemia, ACD, MDS |
| Treatment | Hereditary: Pyridoxine (B6) 200–300 mg/day; Acquired: remove cause (stop alcohol, drugs); Chelation for iron overload; Transfusion support; MDS-type: lenalidomide (del 5q) |
| Blood Transfusion | PRBCs if symptomatic anemia; avoid iron supplementation |
| OPD | Monitor ferritin; pyridoxine response assessment |
| ICU | Transfusion support; manage iron overload complications |
PART B: CLOTTING & BLEEDING DISORDERS (Types 10–17)
10. HEMOPHILIA A (Factor VIII Deficiency)
| Feature | Detail |
|---|---|
| Type | X-linked recessive bleeding disorder |
| Findings | Hemarthrosis (knees, ankles, elbows), muscle hematomas, prolonged bleeding after trauma/surgery, intracranial hemorrhage in severe disease |
| Investigations | PT: Normal; aPTT: ↑↑; Bleeding time: Normal; Factor VIII level ↓↓ (severe <1%, moderate 1–5%, mild 5–40%); VWF antigen: Normal |
| Diagnosis Summary | Prolonged aPTT + normal PT + ↓Factor VIII activity |
| Differential Diagnosis | Hemophilia B (Factor IX), Von Willebrand disease, Factor XI deficiency |
| Treatment | Factor VIII concentrate (recombinant preferred); Prophylactic infusions in severe disease; Emicizumab (subcutaneous, bispecific antibody for Hemophilia A ± inhibitors); DDAVP (desmopressin) for mild disease; Tranexamic acid; Cryoprecipitate (contains FVIII) |
| Contraindications | Avoid IM injections, NSAIDs, aspirin; avoid DDAVP in severe Hemophilia A (insufficient FVIII); avoid arterial punctures |
| Blood Transfusion | Fresh Frozen Plasma (FFP): if factor concentrate unavailable; Cryoprecipitate (FVIII, fibrinogen, VWF, FXIII) |
| OPD | Comprehensive hemophilia center care; physiotherapy; dental care; vaccination (Hep A, Hep B); monitor for inhibitors (FVIII inhibitor titer) |
| ICU | Life-threatening bleed: high-dose factor VIII + ByPass agents (FEIBA or recombinant FVIIa) if inhibitors present; neurosurgical consultation for ICH |
11. HEMOPHILIA B (Factor IX Deficiency — Christmas Disease)
| Feature | Detail |
|---|---|
| Type | X-linked recessive |
| Investigations | PT: Normal; aPTT ↑; Factor IX ↓; Distinguish from Hemophilia A by mixing studies and factor assay |
| Treatment | Factor IX concentrate (recombinant/plasma-derived); Fitusiran (RNA interference therapy); No DDAVP response; Gene therapy (etranacogene dezaparvovec — approved) |
| Blood Transfusion | FFP contains Factor IX; factor concentrate preferred |
| OPD/ICU | Same as Hemophilia A; prophylaxis with extended half-life products |
12. VON WILLEBRAND DISEASE (VWD)
| Feature | Detail |
|---|---|
| Type | Most common inherited bleeding disorder; VWF deficiency or dysfunction |
| Classification | Type 1 (partial quantitative ↓), Type 2 (qualitative defect), Type 3 (complete absence — most severe) |
| Findings | Mucocutaneous bleeding: epistaxis, gum bleeding, menorrhagia, easy bruising; post-surgical bleeding |
| Investigations | Bleeding time ↑; aPTT ↑ (Type 3); VWF antigen ↓; VWF activity (Ristocetin cofactor assay) ↓; Factor VIII ↓ (especially Type 3); Ristocetin-induced platelet aggregation (RIPA) |
| Diagnosis Summary | ↓VWF antigen + ↓VWF activity + clinical bleeding history |
| Differential Diagnosis | Platelet dysfunction, Hemophilia A, Ehlers-Danlos syndrome |
| Treatment | Type 1: DDAVP (desmopressin) 0.3 mcg/kg IV/SC; Tranexamic acid; Types 2B/3: VWF concentrate (Humate-P, Wilate); Recombinant VWF (Vonvendi); Avoid DDAVP in Type 2B (worsens thrombocytopenia) |
| Contraindications | DDAVP: avoid in Type 2B, hyponatremia risk, history of seizures |
| Blood Transfusion | Cryoprecipitate (rich in VWF); Platelet transfusion in Type 2B |
| OPD | Avoid NSAIDs, aspirin; carry medical alert; pre-procedural planning |
| ICU | Major bleeding: VWF concentrate; hemostatic assessment |
13. DISSEMINATED INTRAVASCULAR COAGULATION (DIC)
| Feature | Detail |
|---|---|
| Type | Acquired syndrome of widespread intravascular clotting with secondary fibrinolysis |
| Causes | Sepsis (most common), obstetric catastrophes (placental abruption, amniotic fluid embolism, IUFD), trauma, burns, malignancy (APML), transfusion reaction, snake bite |
| Findings | Simultaneous bleeding (ooze from IV sites, GI, pulmonary) and thrombosis; purpura fulminans; acral cyanosis; organ failure |
| Investigations | PT ↑; aPTT ↑; Fibrinogen ↓↓ (<1.5 g/L); D-dimer ↑↑; FDPs ↑; Platelets ↓↓; Peripheral smear: schistocytes; ISTH DIC Score ≥5: overt DIC |
| Diagnosis Summary | Clinical context + ISTH DIC score (PT, fibrinogen, D-dimer, platelets) |
| Differential Diagnosis | TTP, HUS, severe liver disease, massive transfusion, primary fibrinolysis |
| Treatment | Treat underlying cause (most important); Bleeding-dominant: FFP (replaces all coagulation factors); Cryoprecipitate (fibrinogen <1.5 g/L); Platelet transfusion (if <50×10⁹/L + bleeding); Thrombosis-dominant: Low-dose heparin; APML-DIC: ATRA + aggressive FFP/cryoprecipitate |
| Contraindications | Heparin contraindicated in bleeding-dominant DIC; avoid routine FFP use in thrombotic DIC |
| Blood Transfusion | FFP 15 mL/kg; Cryoprecipitate 1–1.5 units/10 kg; Platelets if <50; PRBCs for anemia |
| ICU | Mandatory ICU admission; manage multiorgan failure; obstetric DIC: deliver fetus; vasopressors; dialysis for renal failure |
14. IMMUNE THROMBOCYTOPENIC PURPURA (ITP)
| Feature | Detail |
|---|---|
| Type | Autoimmune destruction of platelets (anti-GPIIb/IIIa antibodies) |
| Classification | Primary vs Secondary (SLE, HIV, HCV, H. pylori, drugs); Acute (children post-viral) vs Chronic (adults) |
| Findings | Petechiae, purpura, epistaxis, gum bleeding, menorrhagia; NO splenomegaly (unlike hypersplenism); Normal WBC/RBC |
| Investigations | CBC: isolated thrombocytopenia (platelets <100×10⁹/L); Normal PT/aPTT; Peripheral smear: large platelets, no schistocytes; Bone marrow (if atypical): ↑megakaryocytes; H. pylori testing; HIV, HCV serology |
| Diagnosis Summary | Isolated thrombocytopenia + exclusion of other causes |
| Differential Diagnosis | TTP, HUS, drug-induced thrombocytopenia (heparin-HITT), pseudothrombocytopenia, DIC, bone marrow failure |
| Treatment | Platelets >30 with no bleeding: observe; First-line: Prednisolone 1 mg/kg/day; IVIG (0.4 g/kg × 5 days or 1 g/kg × 2 days); Anti-D immunoglobulin; Second-line: Rituximab; Thrombopoietin receptor agonists (TPO-RA: romiplostim, eltrombopag); Third-line: Splenectomy; Fostamatinib |
| Contraindications | Avoid live vaccines during immunosuppression; platelet transfusions have limited benefit (destroyed rapidly) — reserve for life-threatening bleeding |
| Blood Transfusion | Platelets only for active life-threatening bleeding (ICH, surgery); target >50×10⁹/L pre-procedure, >100 pre-neuro surgery |
| OPD | Platelet count monitoring; avoid NSAIDs/aspirin; H. pylori eradication; pre-procedure planning |
| ICU | ICH: IVIG + platelet transfusion + neurosurgical evaluation; High-dose methylprednisolone; emergency splenectomy may be considered |
15. THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP)
| Feature | Detail |
|---|---|
| Type | Thrombotic microangiopathy (TMA); deficiency of ADAMTS13 (VWF-cleaving protease) |
| Classic Pentad | Thrombocytopenia + MAHA (microangiopathic hemolytic anemia) + Fever + Renal failure + Neurological symptoms |
| Findings | Purpura, fluctuating neurological deficits, confusion, seizures, renal impairment, fever |
| Investigations | CBC: ↓Platelets; ↓Hb; Peripheral smear: schistocytes (fragmented RBCs) — KEY; LDH ↑↑; Indirect bilirubin ↑; Coombs test NEGATIVE; ADAMTS13 activity <10% (acquired TTP); Creatinine ↑ |
| Diagnosis Summary | Schistocytes on smear + thrombocytopenia + ↑LDH + negative Coombs + ↓ADAMTS13 |
| Differential Diagnosis | HUS (more renal, less neuro), DIC, HELLP, sepsis, Evans syndrome |
| Treatment | EMERGENCY: Plasma exchange (plasmapheresis) — FIRST-LINE and life-saving; Corticosteroids; Rituximab (anti-CD20, reduces relapses); Caplacizumab (anti-VWF nanobody — blocks platelet aggregation); Immunosuppression |
| Contraindications | NEVER transfuse platelets in TTP (worsens thrombosis — "add fuel to fire"); avoid platelet transfusion except life-threatening hemorrhage |
| Blood Transfusion | PRBCs for severe anemia; NO platelets; plasma transfusion as bridge to plasmapheresis |
| ICU | Mandatory ICU; daily plasma exchange until remission (platelets >150 × 2 days + ↑ADAMTS13); dialysis if needed |
16. HEPARIN-INDUCED THROMBOCYTOPENIA (HIT)
| Feature | Detail |
|---|---|
| Type | Immune-mediated drug reaction causing thrombocytopenia AND thrombosis paradox |
| Mechanism | IgG antibodies against heparin-PF4 complex → platelet activation → thrombosis |
| Findings | Platelet drop >50% from baseline (5–14 days after heparin); thrombosis (DVT, PE, arterial); skin necrosis at injection sites; HIT Type II (clinically significant) |
| Investigations | 4T Score (clinical pre-test probability); Platelet factor 4 (PF4-heparin) ELISA (high sensitivity); Serotonin release assay (SRA) — gold standard; CBC; Coagulation screen |
| Diagnosis Summary | 4T score ≥6 + positive PF4/heparin antibody + clinical thrombocytopenia |
| Differential Diagnosis | Drug-induced thrombocytopenia (non-immune), sepsis-related, DIC, TTP |
| Treatment | STOP ALL heparin immediately; Start alternative anticoagulation: Argatroban (preferred in renal failure), Bivalirudin, Fondaparinux, or Danaparoid; Rivaroxaban/apixaban after platelet recovery; Do NOT start warfarin until platelets recover (risk of venous limb gangrene) |
| Contraindications | Heparin (all formulations including LMWH and catheter flushes) absolutely contraindicated; Warfarin contraindicated while thrombocytopenic |
| Blood Transfusion | Platelet transfusion contraindicated (worsens thrombosis) |
| ICU | Argatroban infusion with aPTT monitoring; DVT/PE management; limb salvage |
17. THROMBOPHILIA (Hypercoagulable States)
| Feature | Detail |
|---|---|
| Type | Inherited or acquired predisposition to thrombosis |
| Causes | Inherited: Factor V Leiden (most common), Prothrombin G20210A, Protein C/S deficiency, Antithrombin III deficiency, MTHFR; Acquired: Antiphospholipid syndrome (APS), malignancy, pregnancy, OCP, nephrotic syndrome |
| Findings | Recurrent DVT/PE, arterial thrombosis (APS), pregnancy loss, Budd-Chiari syndrome, cerebral venous sinus thrombosis |
| Investigations | Factor V Leiden PCR; Prothrombin gene mutation; Protein C, S, AT-III levels (check OFF anticoagulation); Antiphospholipid antibodies (anti-cardiolipin IgG/IgM, anti-β2GP1, lupus anticoagulant) — confirm ≥12 weeks apart |
| Diagnosis Summary | Thrombophilia screen + clinical context; APS: thrombosis + persistent antiphospholipid antibodies |
| Differential Diagnosis | Malignancy-associated thrombosis, HITT, PNH |
| Treatment | Anticoagulation (LMWH, DOAC, warfarin based on type); APS: warfarin (target INR 2–3, or 3–4 for arterial thrombosis); lifelong therapy for recurrent events |
| Contraindications | Rivaroxaban/apixaban not recommended in high-risk APS (triple positivity) — use warfarin |
| OPD | Thrombophilia screening; education on triggers; pregnancy management (LMWH) |
| ICU | Massive thrombosis → thrombolysis; catheter-directed therapy |
PART C: MALIGNANT BLOOD DISORDERS (Types 18–24)
18. ACUTE MYELOID LEUKEMIA (AML)
| Feature | Detail |
|---|---|
| Findings | Fatigue, pallor, fever, bleeding (DIC in APML), bone pain, gum hypertrophy (M4/M5), leukostasis (WBC >100,000) |
| Investigations | CBC: anemia, thrombocytopenia, ↑WBC (blasts); Peripheral smear + bone marrow: ≥20% blasts; Auer rods (pathognomonic); Cytogenetics: t(15;17) = APML [good prognosis]; t(8;21), inv(16) [good]; FLT3, NPM1, IDH1/2 mutations; LDH ↑; Uric acid ↑ |
| Diagnosis Summary | ≥20% myeloblasts in marrow + cytogenetics/molecular markers |
| Differential Diagnosis | ALL, AML-MRC (myelodysplasia-related changes), blast crisis of CML, MDS |
| Treatment | Standard: "7+3" induction (cytarabine 7 days + daunorubicin/idarubicin 3 days); APML [M3]: ATRA + ATO (arsenic trioxide) ± chemotherapy; FLT3+: Midostaurin; IDH1/2: Ivosidenib/Enasidenib; Consolidation: Cytarabine; Eligible: allogeneic HSCT |
| Contraindications | Avoid high-dose cytarabine in poor renal function; ATRA contraindicated in leukocytosis without cover (differentiation syndrome risk) |
| Blood Transfusion | RBCs: Hb <8 g/dL; Platelets: <10×10⁹/L (prophylactic) or <50 (bleeding); Irradiated, CMV-negative products; FFP/cryoprecipitate for DIC (APML) |
| ICU | Leukostasis: hydroxyurea, leukapheresis; tumor lysis syndrome (TLS): aggressive hydration, allopurinol/rasburicase; DIC management; Neutropenic fever protocol; differentiation syndrome (APML): dexamethasone |
19. ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)
| Feature | Detail |
|---|---|
| Findings | Children most common; lymphadenopathy, hepatosplenomegaly, CNS symptoms (headache, cranial nerve palsy), mediastinal mass (T-cell ALL), bone pain |
| Investigations | Bone marrow: ≥20% lymphoblasts; Immunophenotyping: B-ALL (CD10, CD19, CD20, TdT+) vs T-ALL (CD3, CD5, TdT+); Cytogenetics: Philadelphia chromosome t(9;22) [poor prognosis]; t(12;21) ETV6-RUNX1 [good]; Hyperdiploidy [good]; BCR-ABL molecular testing; CSF for CNS involvement |
| Diagnosis Summary | Lymphoblasts ≥20% + immunophenotyping + cytogenetics |
| Differential Diagnosis | AML, CLL, lymphoma with leukemic phase, viral lymphocytosis |
| Treatment | Multi-phase: Induction (vincristine, prednisolone, asparaginase, daunorubicin) → Consolidation → Maintenance (6-mercaptopurine, methotrexate); CNS prophylaxis (intrathecal methotrexate); Ph+ ALL: add Imatinib/Dasatinib; Refractory/relapsed: Blinatumomab (BiTE), Inotuzumab ozogamicin; HSCT |
| Contraindications | Asparaginase: avoid in pancreatitis, hepatic failure, prior hypersensitivity |
| Blood Transfusion | Same as AML; CNS disease: avoid platelet drops |
| ICU | TLS prevention; superior vena cava syndrome (T-ALL mediastinal mass); CNS leukemia: dexamethasone + intrathecal therapy |
20. CHRONIC MYELOID LEUKEMIA (CML)
| Feature | Detail |
|---|---|
| Findings | Massive splenomegaly (most prominent), fatigue, night sweats, weight loss, leukocytosis, basophilia; Blast crisis = acute leukemia |
| Investigations | CBC: ↑↑WBC (20,000–500,000), basophilia, eosinophilia; Peripheral smear: full spectrum of myeloid cells; Bone marrow: hypercellular; Philadelphia chromosome t(9;22)/BCR-ABL FISH/PCR — DIAGNOSTIC; LAP score ↓ (distinguishes from leukemoid reaction) |
| Diagnosis Summary | BCR-ABL fusion gene (Philadelphia chromosome) |
| Differential Diagnosis | Leukemoid reaction (LAP ↑), PV, ET, myelofibrosis |
| Treatment | Tyrosine kinase inhibitors (TKIs): Imatinib (1st line), Dasatinib, Nilotinib (2nd gen); Ponatinib (T315I mutation — resistant CML); Blast crisis: intensified chemotherapy + TKI; Curative: Allogeneic HSCT (for blast crisis or TKI failure); Hydroxyurea (cytoreduction before TKI) |
| Contraindications | Imatinib: avoid in pregnancy; nilotinib: QTc prolongation monitoring; dasatinib: pleural effusion risk |
| OPD | BCR-ABL PCR monitoring (major molecular response at 12 months target); CBC; bone density |
| ICU | Leukostasis, blast crisis management; TLS; spleen rupture |
21. CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
| Feature | Detail |
|---|---|
| Findings | Elderly patients; lymphadenopathy, splenomegaly, recurrent infections; Smudge cells on smear; Often asymptomatic (incidental finding) |
| Investigations | CBC: lymphocytosis >5×10⁹/L; Peripheral smear: mature lymphocytes + smudge cells; Immunophenotyping: CD5+, CD19+, CD20 (dim), CD23+; ZAP-70, CD38, IGHV mutation status; Cytogenetics: del(17p)/TP53 mutation (worst prognosis), del(11q), trisomy 12, del(13q) (best prognosis); Coombs test |
| Diagnosis Summary | Persistent lymphocytosis >5×10⁹/L + B-cell immunophenotype (CD5/CD19/CD23+) |
| Differential Diagnosis | Mantle cell lymphoma (CD5+, CD23-), FL with leukemia, hairy cell leukemia |
| Treatment | Watch and wait for Rai 0-II asymptomatic; Symptomatic: Venetoclax (BCL-2 inhibitor) + Obinutuzumab; Ibrutinib (BTK inhibitor); FCR (fludarabine, cyclophosphamide, rituximab) — in IGHV-mutated CLL; HSCT for refractory disease; Autoimmune hemolysis: steroids |
| Contraindications | Fludarabine: avoid in poor performance status/Coombs+ (worsens AIHA); Ibrutinib: anticoagulant interaction risk |
| Blood Transfusion | AIHA: washed/irradiated RBCs; ITP: corticosteroids preferred over platelet transfusion |
| OPD | Infection surveillance; PCP prophylaxis; vaccination (pneumococcal, flu); IVIG for recurrent infections |
| ICU | Richter transformation (aggressive DLBCL): chemotherapy; sepsis |
22. MULTIPLE MYELOMA
| Feature | Detail |
|---|---|
| Findings | CRAB criteria: Calcium ↑, Renal failure, Anemia, Bone lesions (lytic); Recurrent infections (hypogammaglobulinemia); Hyperviscosity syndrome; Back pain; AL amyloidosis |
| Investigations | CBC: normocytic anemia; Serum protein electrophoresis (SPEP): M-protein spike; Serum free light chains (FLC) ratio; Bence-Jones protein (urine); Bone marrow: ≥10% clonal plasma cells; Skeletal survey/PET-CT/MRI: lytic lesions; Beta-2 microglobulin (prognosis); LDH; Creatinine; Calcium; FISH: del(17p), t(4;14), t(14;16) — high risk |
| Diagnosis Summary | ≥10% clonal plasma cells + CRAB criteria OR biomarkers (>60% plasma cells, FLC ratio >100, >1 focal lesion) |
| Differential Diagnosis | MGUS, smoldering myeloma, Waldenstrom macroglobulinemia, POEMS, metastatic carcinoma, plasmacytoma |
| Treatment | Transplant-eligible: VRd (bortezomib + lenalidomide + dexamethasone) × 4 cycles → autologous HSCT → lenalidomide maintenance; Transplant-ineligible: VRd or DRd (daratumumab + lenalidomide + dex); Relapsed/refractory: Daratumumab, carfilzomib, pomalidomide, selinexor, belantamab, bispecific antibodies (teclistamab); Supportive: Zoledronic acid (bone protection), erythropoietin, G-CSF, prophylactic LMWH (with lenalidomide), calcium/Vit D |
| Contraindications | Thalidomide/lenalidomide: teratogenic (contraception mandatory); bortezomib: peripheral neuropathy monitoring; bisphosphonates: avoid if CrCl <30 (use denosumab) |
| Blood Transfusion | RBCs for symptomatic anemia; ESAs to reduce transfusion burden |
| OPD | M-protein monitoring; free light chains; renal function; bone density |
| ICU | Hypercalcemia crisis: IV fluids + bisphosphonates + calcitonin; Hyperviscosity: plasmapheresis; Spinal cord compression: emergency dexamethasone + radiotherapy/surgery |
23. HODGKIN LYMPHOMA (HL)
| Feature | Detail |
|---|---|
| Findings | Young adults; painless lymphadenopathy (cervical most common), B symptoms (fever, night sweats, weight loss >10%), Reed-Sternberg cells ("owl-eye"), mediastinal mass, alcohol-induced pain in nodes, pruritus |
| Investigations | Lymph node biopsy (excisional): Reed-Sternberg cells (CD15+, CD30+, CD45−) — DIAGNOSTIC; CBC: normocytic anemia, eosinophilia; ESR ↑; LDH ↑; PET-CT scan (staging and response assessment); Bone marrow biopsy; Staging: Ann Arbor I–IV |
| Diagnosis Summary | Excisional lymph node biopsy + RS cell immunophenotype |
| Differential Diagnosis | Non-Hodgkin lymphoma, infectious mononucleosis, sarcoidosis, reactive lymphadenopathy, metastatic cancer |
| Treatment | Early stage (I-IIA): ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) × 2–4 cycles + radiotherapy; Advanced (III-IV): ABVD × 6 cycles or BV-AVD (brentuximab vedotin); Refractory/relapse: salvage chemo (ICE, DHAP) → autologous HSCT; Nivolumab/Pembrolizumab (PD-1 inhibitors) |
| Contraindications | Bleomycin: avoid with low DLCO or pulmonary disease; Adriamycin: limit cumulative dose (cardiac toxicity); avoid radiotherapy to heart without shielding |
| Blood Transfusion | Irradiated blood products to prevent transfusion-associated GvHD in immunosuppressed patients |
| OPD | PET-CT response assessment after 2 cycles; long-term surveillance for secondary malignancies (breast cancer post-mantle RT), cardiac disease, pulmonary toxicity |
| ICU | Superior vena cava obstruction: steroids + chemo; massive mediastinal disease and airway compromise |
24. NON-HODGKIN LYMPHOMA (NHL)
| Feature | Detail |
|---|---|
| Findings | Diverse group; B or T cell; aggressive (DLBCL, Burkitt) vs indolent (FL, marginal zone); extranodal involvement (GI, CNS, skin); B symptoms |
| Investigations | Excisional biopsy + immunohistochemistry; Flow cytometry; Cytogenetics: t(14;18) = follicular; t(8;14) = Burkitt; t(11;14) = mantle cell; PET-CT staging; LDH; IPI score; BCL-2, BCL-6, MYC (double/triple hit) |
| Diagnosis Summary | Tissue biopsy + immunophenotyping + molecular/cytogenetics |
| Differential Diagnosis | HL, CLL, reactive lymphadenopathy, metastatic cancer |
| Treatment | DLBCL: R-CHOP (rituximab + cyclophosphamide + doxorubicin + vincristine + prednisolone) × 6 cycles; Follicular: R-bendamustine or watch-and-wait; Burkitt: intensive regimens (CODOX-M/IVAC) + prophylactic intrathecal; CNS prophylaxis; CAR-T cell therapy: for relapsed/refractory DLBCL; Bispecifics: Mosunetuzumab |
| Contraindications | Rituximab: Hep B reactivation (screen and prophylax); doxorubicin: cardiac toxicity monitoring |
| Blood Transfusion | Irradiated products; RBCs for anemia; TLS prevention |
| ICU | TLS protocol; Burkitt's: emergency chemo + massive TLS prevention; CNS lymphoma: high-dose methotrexate |
PART D: MYELOPROLIFERATIVE & MARROW DISORDERS (Types 25–27)
25. POLYCYTHEMIA VERA (PV)
| Feature | Detail |
|---|---|
| Type | Myeloproliferative neoplasm; clonal ↑RBC mass (JAK2 mutation) |
| Findings | Plethora (ruddy complexion), headache, dizziness, pruritus after bathing (aquagenic), splenomegaly, hypertension, thrombosis (stroke, Budd-Chiari, portal vein), gout |
| Investigations | CBC: Hb >16.5 (men)/16 (women), ↑Hematocrit, ↑WBC, ↑platelets; JAK2 V617F mutation (>95% positive) — KEY; EPO level ↓↓; Bone marrow: hypercellular, trilineage hyperplasia; Serum ferritin; O2 saturation |
| Diagnosis Summary | WHO 2022: Major criteria — ↑Hb/Hct + trilineage BM hyperplasia + JAK2 mutation; EPO ↓ |
| Differential Diagnosis | Secondary polycythemia (↑EPO — hypoxia, EPO-secreting tumor), relative polycythemia, essential thrombocythemia |
| Treatment | Low-risk (<60 yrs, no thrombosis): Phlebotomy (target Hct <45%) + aspirin 75–100 mg; High-risk: Hydroxyurea + phlebotomy + aspirin; Refractory: Ruxolitinib (JAK1/2 inhibitor); Anagrelide (thrombocytosis); Interferon-alpha |
| Contraindications | Avoid iron supplementation (stimulates erythropoiesis); phlebotomy contraindicated if recent thrombosis or severe cardiopulmonary disease |
| OPD | Hematocrit monitoring; thrombosis risk stratification; manage CV risk factors |
| ICU | Thrombotic crisis: anticoagulation; Budd-Chiari: TIPS; hyperviscosity: urgent phlebotomy |
26. ESSENTIAL THROMBOCYTHEMIA (ET)
| Feature | Detail |
|---|---|
| Type | MPN; ↑platelets (>450×10⁹/L) due to clonal megakaryocyte proliferation |
| Findings | Often asymptomatic (incidental); headaches, visual changes, thrombosis (paradoxically also bleeding with extreme thrombocytosis), erythromelalgia (burning pain of hands/feet) |
| Investigations | CBC: platelets >450×10⁹/L; JAK2 (50%), CALR (25%), MPL (5%) mutations; Peripheral smear: large platelets; Bone marrow: megakaryocyte proliferation; Exclude reactive causes (iron deficiency, infection, splenectomy) |
| Diagnosis Summary | Platelet count >450 + BM findings + JAK2/CALR/MPL mutation |
| Differential Diagnosis | PV, reactive thrombocytosis, CML, MF, iron deficiency |
| Treatment | Low-risk: Aspirin only; High-risk (>60, prior thrombosis, cardiovascular risk, extreme platelets): Hydroxyurea (1st line); Anagrelide (2nd line); Interferon-alpha; Ruxolitinib for refractory |
| Contraindications | Aspirin paradoxically increases bleeding if platelets >1,000×10⁹/L; anagrelide: fluid retention, cardiac effects |
| OPD | Platelet monitoring; mutation testing; transformation surveillance (MF, AML) |
27. MYELODYSPLASTIC SYNDROME (MDS)
| Feature | Detail |
|---|---|
| Type | Clonal stem cell disorder with ineffective hematopoiesis and risk of AML transformation |
| Classification | IPSS-R: very low, low, intermediate, high, very high risk |
| Findings | Cytopenias (anemia most common), recurrent infections, bleeding, dysplastic blood cells |
| Investigations | CBC: cytopenias; Peripheral smear: hyposegmented neutrophils (pseudo-Pelger-Huët), dysplastic RBCs; Bone marrow: dysplasia in ≥10% of one or more cell lines, ringed sideroblasts; Cytogenetics: del(5q) [best prognosis], monosomy 7 [poor]; Molecular: SF3B1 (ringed sideroblasts), TP53, TET2; Ferritin (transfusion iron overload) |
| Diagnosis Summary | Cytopenias + morphological dysplasia + cytogenetics/molecular profile |
| Differential Diagnosis | B12/folate deficiency, aplastic anemia, AML, CML, ACD |
| Treatment | Low-risk: ESAs (darbepoetin); Lenalidomide (del 5q); luspatercept (SF3B1+); supportive transfusions; chelation; High-risk: Azacitidine (hypomethylating agent) or decitabine; venetoclax + azacitidine; Curative: Allogeneic HSCT (only) |
| Contraindications | ESAs: avoid if Hb >11 or high-risk MDS; lenalidomide: teratogenic, thrombosis risk |
| Blood Transfusion | Regular RBC transfusions; target Hb >8; iron chelation when ferritin >1000–2500 ng/mL |
| ICU | AML transformation → AML treatment protocols |
PART E: BODY FLUID & TRANSFUSION-RELATED DISORDERS (Types 28–30)
28. PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH)
| Feature | Detail |
|---|---|
| Type | Acquired clonal disorder (PIGA mutation); deficiency of GPI-anchored proteins (CD55, CD59) |
| Findings | Intravascular hemolysis, dark morning urine (hemoglobinuria), thrombosis (atypical sites: hepatic/mesenteric/cerebral veins), cytopenias, aplastic anemia overlap |
| Investigations | Flow cytometry: absent CD55/CD59 on RBCs and granulocytes — GOLD STANDARD (>1% GPI-deficient cells); Ham's test/sucrose lysis (historical); CBC: hemolytic anemia; LDH ↑↑; Haptoglobin ↓; DAT: negative; Urine hemosiderin; D-dimer (thrombosis) |
| Diagnosis Summary | Flow cytometry showing absent CD55/CD59 |
| Differential Diagnosis | AIHA, aplastic anemia, MDS, other intravascular hemolysis |
| Treatment | Eculizumab (anti-C5 complement inhibitor) — transforms disease; Ravulizumab (every 8 weeks); Iptacopan (oral, factor B inhibitor); Anticoagulation (long-term) for thrombosis; Iron/folate supplementation; Curative: Allogeneic HSCT |
| Contraindications | Eculizumab: mandatory meningococcal vaccination before starting; avoid in unvaccinated patients; |
| Blood Transfusion | Washed RBCs preferred (to minimize complement activation from donor plasma); PRBCs for severe anemia |
| OPD | Complement inhibitor monitoring; infection surveillance; meningococcal prophylaxis (penicillin); thrombosis surveillance |
| ICU | Thrombotic crisis: anticoagulation + complement inhibitor; Budd-Chiari syndrome management |
29. BLOOD TRANSFUSION REACTIONS (Transfusion Medicine)
| Feature | Detail |
|---|---|
| Types & Mechanisms | Acute Hemolytic (AHTR): ABO incompatibility → complement activation → intravascular hemolysis; FNHTR: recipient antibodies against donor leukocytes/cytokines; Allergic: IgE-mediated (urticaria); Anaphylaxis: anti-IgA in IgA-deficient recipient; TACO (Transfusion-Associated Circulatory Overload): volume overload; TRALI (Transfusion-Related Acute Lung Injury): anti-HLA/anti-neutrophil antibodies from donor; Transfusion-associated GvHD: donor T-cells vs recipient; Delayed hemolytic (DHTR): anamnestic antibody response (days 3–14) |
| Findings/Features | AHTR: fever, chills, pain, shock, hemoglobinuria, renal failure; TACO: dyspnea, hypertension, pulmonary edema; TRALI: dyspnea, hypoxia, bilateral pulmonary infiltrates WITHOUT hypertension (unlike TACO); FNHTR: fever, chills, no hemolysis; Anaphylaxis: urticaria, bronchospasm, shock |
| Investigations | Repeat cross-match; DAT (Coombs); Blood cultures (bacterial contamination); Urine hemoglobin; Chest X-ray; BNP (TACO); CBC; Coagulation; Recipient/donor anti-HLA antibodies (TRALI) |
| Diagnosis Summary | Clinical presentation timing + type of blood product + laboratory markers |
| Treatment | Stop transfusion immediately (all reactions); Maintain IV access; Supportive care; AHTR: IV fluids + mannitol/furosemide (renal protection), treat DIC; TACO: diuresis (furosemide); TRALI: respiratory support (O2/mechanical ventilation) — NO diuretics; Anaphylaxis: epinephrine + antihistamines; FNHTR: antipyretics; GvHD: irradiated blood products prevention |
| Prevention/Contraindications | Type and screen/cross-match before every transfusion; irradiated blood (prevent GvHD); leukoreduced blood (prevent FNHTR, CMV); washed RBCs (IgA deficiency); CMV-negative blood (immunosuppressed) |
| OPD | Transfusion history documentation; alloantibody identification card |
| ICU | TRALI: ICU mandatory; mechanical ventilation; rule out TACO; supportive management |
30. SEPSIS-ASSOCIATED HEMATOLOGICAL DYSFUNCTION (Critical Care Hematology)
| Feature | Detail |
|---|---|
| Type | Hematological abnormalities complicating sepsis/critical illness |
| Findings | Anemia (multifactorial — phlebotomy, hemolysis, inflammation, bone marrow suppression); Thrombocytopenia (DIC, sepsis itself, drug-induced, HIT); Leukopenia or leukocytosis; DIC; Venous thromboembolism; Immune dysregulation |
| Investigations | CBC with differential; PT, aPTT, fibrinogen, D-dimer, FDPs; Peripheral smear (schistocytes → TMA/DIC); Blood cultures × 2; Serum ferritin (hyperferritinemia in HLH); Bone marrow in suspected HLH; HIT antibody (if heparin-treated and falling platelets); ADAMTS13 (if TTP suspected) |
| Diagnosis Summary | Clinical context + CBC trends + coagulation screen; consider HLH score (HScore) in refractory cytopenia |
| Differential Diagnosis | TTP, HUS, DIC, HIT, HLH (hemophagocytic lymphohistiocytosis), aplastic anemia, drug-induced |
| Treatment | Anemia: Restrictive transfusion strategy (Hb <7 g/dL; Hb <8 in cardiac); Thrombocytopenia: treat underlying cause; platelet transfusion if <10 (prophylactic) or <50 (bleeding/surgery); DIC: treat sepsis source (antibiotics, source control) + blood products; VTE prophylaxis: LMWH/UFH (unless bleeding); HLH: dexamethasone + etoposide ± cyclosporine ± anakinra |
| Contraindications | Avoid liberal transfusion strategy (↑mortality, TACO, TRALI); avoid platelets in TTP/HIT; avoid heparin in HIT |
| Blood Transfusion | Restrictive strategy (Hb threshold 7 g/dL) per TRICC, TRISS trials; higher threshold (8–9) in cardiac/neurological patients; PRBCs only (not whole blood in routine ICU care) |
| ICU | Multidisciplinary; daily CBC and coagulation monitoring; source control paramount; APACHE/SOFA scoring; coagulopathy management integral to sepsis bundles |
🔬 QUICK REFERENCE: TYPES OF ANEMIA
| Category | Type | Key Feature | MCV |
|---|---|---|---|
| Microcytic | Iron Deficiency | ↓Ferritin, ↑TIBC | <80 fL |
| Thalassemia | Normal/↑RBC count, Hb electrophoresis | <80 fL | |
| ACD | ↑Ferritin, ↓TIBC | Normal/Low | |
| Sideroblastic | Ring sideroblasts, ↑Fe | Low/Normal | |
| Lead Poisoning | Basophilic stippling | Low | |
| Macrocytic | B12 Deficiency | SACD, hypersegmented neutrophils | >100 fL |
| Folate Deficiency | No neuro features | >100 fL | |
| Liver Disease/Alcohol | Target cells | >100 fL | |
| Hypothyroidism | Clinical hypothyroidism | >100 fL | |
| MDS | Dysplastic cells, BM changes | Normal/High | |
| Normocytic | Aplastic Anemia | Pancytopenia, hypocellular BM | 80–100 fL |
| Hemolytic Anemia | ↑LDH, ↑retics, ↓haptoglobin | Normal/High (retics) | |
| Acute Blood Loss | Clinical hemorrhage | Normal | |
| Chronic Kidney Disease | ↓EPO | Normal | |
| Hypothyroidism (early) | TSH ↑ | Normal |
💉 BLOOD TRANSFUSION — KEY PRINCIPLES
| Indication | Product | Threshold |
|---|---|---|
| Symptomatic anemia | Packed RBCs (PRBCs) | Hb <7 (ICU/general); <8 (cardiac/post-op) |
| Thrombocytopenia | Platelets | <10 (prophylactic); <50 (bleeding/procedure); <100 (neuro-surgery) |
| Coagulopathy/DIC | Fresh Frozen Plasma (FFP) | PT/INR >1.5× normal; fibrinogen <1.5 g/L |
| Fibrinogen deficiency | Cryoprecipitate | Fibrinogen <1.5 g/L; factor VIII/XIII, VWF |
| Hemophilia A/VWD | Factor concentrates | Targeted replacement |
| Massive transfusion | 1:1:1 ratio (RBC:FFP:Platelets) | Trauma/obstetric hemorrhage |
🏥 ICU vs OPD MANAGEMENT FRAMEWORK
| Disease | OPD Focus | ICU Triggers |
|---|---|---|
| Iron Deficiency | Oral iron, diet, treat source | Hb <7 with hemodynamic instability |
| Aplastic Anemia | Cyclosporine monitoring, infection prophylaxis | Neutropenic sepsis, severe pancytopenia |
| Sickle Cell | Hydroxyurea, vaccines, transcranial Doppler | Acute chest syndrome, stroke, multi-organ failure |
| Thalassemia | Transfusion schedule, chelation | Cardiac siderosis, severe anemia |
| AML/ALL | Outpatient maintenance chemo | Induction, TLS, neutropenic fever, DIC |
| Multiple Myeloma | VRd cycles, monitoring, bisphosphonates | Hypercalcemia, cord compression, renal failure |
| TTP | Follow ADAMTS13 levels, rituximab | Emergency plasmapheresis |
| DIC | Treat underlying disease | All DIC is ICU level care |
| ITP | Steroids, IVIG, TPO-RA | ICH, life-threatening bleeding |
| PNH | Eculizumab infusions, anticoagulation | Thrombotic crisis, Budd-Chiari |
Sources: Goldman-Cecil Medicine; Tintinalli's Emergency Medicine; Henry's Clinical Diagnosis and Management by Laboratory Methods; Tietz Textbook of Laboratory Medicine; WHO Classification of Haematolymphoid Tumours (2022); British Society for Haematology GuidelinesHere is a complete summary of what this reference covers:
📋 WHAT'S COVERED — 30 DISEASES INDEX
Part A — Anemias (1–9)
- Iron Deficiency Anemia
- Vitamin B12 Deficiency (Megaloblastic)
- Folate Deficiency Anemia
- Aplastic Anemia
- Hemolytic Anemia
- Sickle Cell Disease
- Thalassemia (α & β)
- Anemia of Chronic Disease
- Sideroblastic Anemia
Part B — Clotting & Bleeding Disorders (10–17)
- Hemophilia A (Factor VIII deficiency)
- Hemophilia B (Factor IX deficiency)
- Von Willebrand Disease
- DIC (Disseminated Intravascular Coagulation)
- Immune Thrombocytopenic Purpura (ITP)
- Thrombotic Thrombocytopenic Purpura (TTP)
- Heparin-Induced Thrombocytopenia (HIT)
- Thrombophilia / Hypercoagulable States
Part C — Malignant Blood Disorders (18–24)
- Acute Myeloid Leukemia (AML)
- Acute Lymphoblastic Leukemia (ALL)
- Chronic Myeloid Leukemia (CML)
- Chronic Lymphocytic Leukemia (CLL)
- Multiple Myeloma
- Hodgkin Lymphoma
- Non-Hodgkin Lymphoma
Part D — Myeloproliferative & Marrow Disorders (25–27)
- Polycythemia Vera
- Essential Thrombocythemia
- Myelodysplastic Syndrome (MDS)
Part E — Body Fluid & Transfusion-Related (28–30)
- Paroxysmal Nocturnal Hemoglobinuria (PNH)
- Blood Transfusion Reactions (all types)
- Sepsis-Associated Hematological Dysfunction
Each entry includes: type classification · clinical findings · investigations · diagnostic criteria · differential diagnosis · treatment (including latest biologics/targeted agents) · contraindications · blood transfusion indications · OPD management · ICU triggers and protocols. The guide also includes an anemia type quick-reference table and a blood transfusion threshold summary.
20 types blood and body fluid system disease treatment investigation finding differential diagnosis contraindications ICU and OPD and blood transfusion and type of diagnosis summary
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