20 types blood and body fluid system disease treatment investigation finding differential diagnosis contraindications ICU and OPD and blood transfusion and type other plasma RBC WBC platelet nutrition flute of diagnosis summary
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Based on the textbook content retrieved and my comprehensive medical knowledge, here is a detailed clinical reference covering 20 Blood & Body Fluid System Diseases — including treatment, investigations, findings, differential diagnosis, contraindications, ICU/OPD management, blood transfusion, blood products, nutrition, and diagnostic summaries.
🩸 20 Blood & Body Fluid System Diseases — Complete Clinical Reference
1. IRON DEFICIENCY ANEMIA (IDA)
| Domain | Details |
|---|---|
| Investigations | CBC (microcytic hypochromic anemia), serum ferritin ↓, serum iron ↓, TIBC ↑, peripheral smear (pencil cells, target cells), reticulocyte count, stool for occult blood |
| Findings | Hb ↓, MCV <80 fL, MCH <27 pg, RDW ↑, ferritin <12 µg/L |
| Differential Dx | Thalassemia minor, anemia of chronic disease, sideroblastic anemia |
| Treatment | Oral ferrous sulfate 325 mg TDS × 3–6 months; IV iron (ferric carboxymaltose) if oral intolerance; treat underlying cause |
| Contraindications | IV iron contraindicated in acute infection; avoid iron in hemochromatosis |
| Blood Transfusion | Packed RBCs if Hb <7 g/dL or symptomatic (<8 in cardiac disease); transfuse slowly to avoid volume overload |
| Blood Products | Packed RBC (pRBC) |
| OPD Management | Oral iron + dietary advice; follow-up CBC at 4 weeks |
| ICU Considerations | Transfusion threshold Hb <7 g/dL (restrictive strategy); IV iron preferred over transfusion in stable ICU patients |
| Nutrition | Iron-rich foods: red meat, leafy greens, fortified cereals; vitamin C with meals to enhance absorption; avoid tea/coffee with meals |
| Diagnostic Summary | Microcytic hypochromic anemia + ↓ ferritin + ↑ TIBC = IDA until proven otherwise |
2. MEGALOBLASTIC ANEMIA (B12 / Folate Deficiency)
| Domain | Details |
|---|---|
| Investigations | CBC (macrocytic anemia), serum B12, serum folate, homocysteine ↑, methylmalonic acid ↑ (B12 deficiency specific), peripheral smear (hypersegmented neutrophils, macro-ovalocytes), LDH ↑, indirect bilirubin ↑ |
| Findings | MCV >100 fL, pancytopenia possible, Hb ↓ |
| Differential Dx | Liver disease, hypothyroidism, aplastic anemia, drug-induced (methotrexate, hydroxyurea) |
| Treatment | B12 deficiency: cyanocobalamin IM 1000 µg daily × 7 days → weekly × 4 → monthly (lifelong if pernicious anemia). Folate deficiency: folic acid 5 mg daily |
| Contraindications | Never give folate alone without ruling out B12 deficiency (may precipitate subacute combined degeneration of cord) |
| Blood Transfusion | Rarely needed; if necessary, transfuse pRBC slowly (risk of fluid overload with compensated chronic anemia) |
| Blood Products | pRBC if Hb critically low |
| OPD Management | Dietary counseling, treat underlying cause (e.g., pernicious anemia → lifelong B12 injections) |
| ICU Considerations | Monitor potassium after treatment (hypokalemia from rapid cell proliferation) |
| Nutrition | B12: meat, fish, dairy, eggs; Folate: leafy greens, legumes, fortified foods |
| Diagnostic Summary | Macrocytic anemia + hypersegmented neutrophils + ↓ B12/folate = megaloblastic anemia |
3. SICKLE CELL DISEASE (SCD)
| Domain | Details |
|---|---|
| Investigations | Hb electrophoresis (HbSS), CBC, reticulocyte count, peripheral smear (sickle cells, target cells), LDH, bilirubin, renal/liver function, urine (for proteinuria), MRI brain (stroke screening), TCD ultrasound in children |
| Findings | Hb 6–9 g/dL, reticulocytosis, elevated LDH, unconjugated bilirubin ↑, cholelithiasis on imaging |
| Differential Dx | HbSC disease, sickle-beta thalassemia, other hemolytic anemias |
| Treatment | Hydroxyurea (↑ HbF, reduces crises); L-glutamine (antioxidant); crizanlizumab (anti-P selectin, 5 mg/kg IV q4 weeks); voxelotor (HbS polymerization inhibitor, 1500 mg oral daily); pain crises: IV fluids + opioids; HSCT (curative) |
| Contraindications | Hydroxyurea contraindicated in pregnancy; avoid iron supplementation unless deficient |
| Blood Transfusion | Simple transfusion for acute anemia, acute chest syndrome, stroke. Exchange transfusion for severe stroke, multiorgan failure. Not indicated for simple pain crisis. Use ABO/Rh/Kell-matched blood |
| Blood Products | pRBC (leukoreduced, antigen-matched) |
| OPD Management | Hydroxyurea, folic acid 5 mg daily, vaccination (pneumococcal, meningococcal, Hib), penicillin prophylaxis in children, ophthalmology referral |
| ICU Considerations | Acute chest syndrome: O2, incentive spirometry, bronchodilators, antibiotics (ceftriaxone + azithromycin), exchange transfusion, ventilatory support |
| Nutrition | High folate diet; adequate hydration; avoid alcohol and cold exposure (trigger crises) |
| Diagnostic Summary | Hemolytic anemia + vaso-occlusive crises + HbSS on electrophoresis + positive sickling test |
4. THALASSEMIA
| Domain | Details |
|---|---|
| Investigations | CBC (microcytic anemia), Hb electrophoresis (↑ HbA2 in β-thal minor >3.5%), serum ferritin, serum iron (normal or ↑), peripheral smear (target cells, basophilic stippling), genetic testing |
| Findings | MCV ↓, MCH ↓, normal/↑ iron stores, HbF ↑ in thalassemia major |
| Differential Dx | IDA, sideroblastic anemia, hemoglobin E disease |
| Treatment | Thalassemia trait: none needed. Thalassemia major: regular pRBC transfusions (every 2–4 weeks, target Hb 9–10 g/dL pre-transfusion); iron chelation (deferasirox 20–40 mg/kg/day or deferoxamine SC); HSCT (curative) |
| Contraindications | Avoid iron supplementation (iron overload); splenectomy requires vaccination and prophylactic penicillin |
| Blood Transfusion | Chronic transfusion program (leukoreduced, cross-matched pRBC) |
| Blood Products | pRBC (leukoreduced, phenotypically matched for extended antigen typing) |
| OPD Management | Transfusion program, chelation therapy, endocrine monitoring (diabetes, hypothyroidism), cardiac MRI for iron overload |
| ICU Considerations | Cardiac iron overload can cause arrhythmias and heart failure; aggressive chelation and cardiac support |
| Nutrition | Avoid iron-rich diet; low-iron diet; folate supplementation (1 mg/day) |
| Diagnostic Summary | Microcytic anemia + ↑ HbA2 (>3.5%) + normal/↑ ferritin + family history = β-thalassemia |
5. HEMOLYTIC ANEMIA (Autoimmune — AIHA)
| Domain | Details |
|---|---|
| Investigations | Direct Coombs test (DAT) — positive, CBC, reticulocyte count, peripheral smear (spherocytes, schistocytes), LDH ↑, haptoglobin ↓, bilirubin ↑, urine hemoglobin/hemosiderin |
| Findings | Normocytic (or macrocytic due to reticulocytosis), elevated LDH, low haptoglobin |
| Differential Dx | Hereditary spherocytosis, G6PD deficiency, TTP, HUS, mechanical hemolysis |
| Treatment | Warm AIHA: prednisolone 1 mg/kg/day; rituximab if steroid-refractory; splenectomy as second-line. Cold agglutinin disease: avoid cold, rituximab, bendamustine |
| Contraindications | Avoid blood transfusion if possible (cross-matching difficult); avoid triggers (cold, oxidant drugs in G6PD) |
| Blood Transfusion | Use "least incompatible" blood in life-threatening hemolysis; washed pRBC preferred |
| Blood Products | pRBC (washed), FFP rarely needed |
| OPD Management | Steroid taper, monitor CBC weekly initially, folic acid supplementation |
| ICU Considerations | Severe hemolysis with Hb <5 g/dL, cardiac compromise: urgent transfusion + IVIG + plasmapheresis in refractory cases |
| Nutrition | Adequate hydration; folate supplementation |
| Diagnostic Summary | Hemolytic anemia + positive DAT + spherocytes on smear = AIHA |
6. APLASTIC ANEMIA
| Domain | Details |
|---|---|
| Investigations | CBC (pancytopenia), reticulocyte count ↓, bone marrow biopsy (hypocellular marrow <25%), peripheral smear (normocytic, no abnormal cells), flow cytometry (PNH clone), chromosomal breakage studies (Fanconi) |
| Findings | Pancytopenia, ANC <0.5×10⁹/L (severe), platelets <20×10⁹/L, Hb ↓ |
| Differential Dx | Myelodysplastic syndrome (MDS), hypocellular leukemia, PNH, B12/folate deficiency |
| Treatment | Severe aplastic anemia <40 years with matched sibling donor: allogeneic HSCT. Others: anti-thymocyte globulin (ATG) + cyclosporine + eltrombopag (TPO agonist); G-CSF; supportive transfusions |
| Contraindications | Avoid allogeneic HSCT in elderly/comorbid patients; avoid unnecessary transfusions (alloimmunization reduces HSCT success) |
| Blood Transfusion | Threshold: Hb <8 g/dL or symptomatic; platelets <10×10⁹/L (prophylactic) or <20×10⁹/L (with fever/bleeding); use irradiated, leukoreduced products |
| Blood Products | pRBC (irradiated, leukoreduced), platelet concentrates (irradiated), FFP |
| OPD Management | Cyclosporine monitoring, infection prophylaxis (antifungals, antivirals), growth factor support |
| ICU Considerations | Neutropenic fever: broad-spectrum antibiotics (piperacillin-tazobactam ± antifungal); reverse isolation; granulocyte transfusion in refractory infection |
| Nutrition | Neutropenic diet (avoid raw foods, undercooked meat); high-protein, high-calorie diet |
| Diagnostic Summary | Pancytopenia + hypocellular bone marrow biopsy + ↓ reticulocytes = aplastic anemia |
7. POLYCYTHEMIA VERA (PV)
| Domain | Details |
|---|---|
| Investigations | CBC (↑ Hb, ↑ RBC, ↑ WBC, ↑ platelets), JAK2 V617F mutation (>95% positive), EPO level (↓), bone marrow biopsy (hypercellular, trilineage hyperplasia), LDH, uric acid |
| Findings | Hb >16.5 g/dL (men) / >16 g/dL (women), hematocrit >49%/48%, JAK2 mutation positive |
| Differential Dx | Secondary polycythemia (hypoxia, EPO-secreting tumors), apparent polycythemia, essential thrombocythemia |
| Treatment | Phlebotomy (target Hct <45%); low-dose aspirin 75–100 mg daily; hydroxyurea (high-risk patients); ruxolitinib (JAK inhibitor) if refractory |
| Contraindications | Avoid iron supplementation; avoid high-dose aspirin; phlebotomy should be gradual |
| Blood Transfusion | Not indicated; phlebotomy is the primary intervention |
| Blood Products | N/A |
| OPD Management | Regular phlebotomy, CBC monitoring, cardiovascular risk reduction, aspirin |
| ICU Considerations | Thrombotic events (stroke, MI, Budd-Chiari): anticoagulation (LMWH → warfarin); acute phlebotomy if hyperviscosity |
| Nutrition | Adequate hydration; avoid dehydration (increases hyperviscosity risk) |
| Diagnostic Summary | ↑ Hb/Hct + JAK2 V617F + ↓ EPO + bone marrow hypercellularity = PV (WHO criteria) |
8. ESSENTIAL THROMBOCYTHEMIA (ET)
| Domain | Details |
|---|---|
| Investigations | CBC (platelets >450×10⁹/L), JAK2 (50–60%), CALR (20–25%), MPL mutations, bone marrow biopsy (megakaryocytic hyperplasia), peripheral smear |
| Findings | Isolated thrombocytosis, normal Hb and WBC, large/dysplastic megakaryocytes on biopsy |
| Differential Dx | Reactive thrombocytosis (infection, iron deficiency, post-splenectomy), PV, MDS |
| Treatment | Low-risk (<60 years, no thrombosis history): aspirin only. High-risk: hydroxyurea (first-line); anagrelide or interferon-alpha (second-line) |
| Contraindications | Anagrelide contraindicated with cardiac disease; avoid antiplatelet therapy if platelet >1500×10⁹/L (paradoxical bleeding risk due to acquired vWD) |
| Blood Transfusion | Only if hemorrhage or surgery-related blood loss |
| Blood Products | pRBC if bleeding occurs |
| OPD Management | CBC every 3 months, aspirin daily, cardiovascular risk management |
| ICU Considerations | Thrombotic or hemorrhagic emergencies; plateletpheresis for extreme thrombocytosis with symptoms |
| Nutrition | Heart-healthy diet, omega-3 fatty acids, hydration |
| Diagnostic Summary | Isolated platelet >450×10⁹/L + JAK2/CALR mutation + bone marrow megakaryocyte hyperplasia + exclusion of reactive causes = ET |
9. MYELODYSPLASTIC SYNDROME (MDS)
| Domain | Details |
|---|---|
| Investigations | CBC (cytopenias), peripheral smear (dysplastic cells, pseudo-Pelger-Huët, hypogranular neutrophils), bone marrow biopsy (dysplasia ≥10% in ≥1 lineage), cytogenetics (del 5q, monosomy 7), flow cytometry, IPSS-R scoring |
| Findings | Macrocytic or normocytic anemia, neutropenia, thrombocytopenia, ring sideroblasts (if MDS-RS), blast count <20% |
| Differential Dx | Aplastic anemia, AML, megaloblastic anemia, drug-induced cytopenias |
| Treatment | Low-risk: ESAs (erythropoietin), lenalidomide (del 5q), transfusion support. High-risk: azacitidine/decitabine (hypomethylating agents); allogeneic HSCT (young, fit patients) |
| Contraindications | Lenalidomide requires VTE prophylaxis and teratogen precautions; ESAs contraindicated in EPO >500 mIU/mL |
| Blood Transfusion | Chronic transfusion support (threshold Hb <8 g/dL); monitor ferritin (iron chelation if >1000 µg/L) |
| Blood Products | pRBC (leukoreduced); platelet transfusions for bleeding |
| OPD Management | Transfusion program, ESA therapy, growth factors (G-CSF), deferasirox for iron overload |
| ICU Considerations | Transformation to AML; neutropenic sepsis; aggressive antibiotic therapy |
| Nutrition | High-protein diet; folate/B12 supplementation; avoid alcohol |
| Diagnostic Summary | Cytopenia + dysplasia ≥10% in ≥1 cell line + <20% blasts + characteristic cytogenetics = MDS |
10. ACUTE MYELOID LEUKEMIA (AML)
| Domain | Details |
|---|---|
| Investigations | CBC (↑ WBC with blasts, anemia, thrombocytopenia), peripheral smear (Auer rods in myeloblasts), bone marrow biopsy (≥20% blasts), cytogenetics (t(8;21), inv(16), t(15;17) for APL), FLT3/NPM1/IDH1/IDH2 mutations, LFTs, coagulation (DIC screen in APL), LP if CNS involvement suspected |
| Findings | Blasts ≥20% in marrow, Auer rods, DIC (especially APL), hyperleukocytosis |
| Differential Dx | ALL, MDS with excess blasts, blastic phase CML, lymphoma with leukemic phase |
| Treatment | Standard: "7+3" (cytarabine × 7 days + daunorubicin × 3 days). APL: ATRA + arsenic trioxide (ATO) ± chemotherapy. Targeted: midostaurin (FLT3+), enasidenib/ivosidenib (IDH mut), venetoclax + azacitidine (elderly/unfit). Consolidation: HSCT in high-risk |
| Contraindications | ATRA contraindicated in pregnancy (teratogenic); avoid anthracyclines in severe cardiac dysfunction |
| Blood Transfusion | pRBC for Hb <8 g/dL; platelets <10–20×10⁹/L (prophylactic); FFP + cryoprecipitate for DIC (especially APL) |
| Blood Products | pRBC (irradiated, leukoreduced), platelets (irradiated), FFP, cryoprecipitate, fibrinogen concentrate |
| OPD Management | Post-remission consolidation cycles, monitoring for relapse, infection prophylaxis |
| ICU Considerations | Tumor lysis syndrome (TLS): aggressive IV hydration, allopurinol/rasburicase, monitor electrolytes. Neutropenic fever: broad-spectrum antibiotics. Leukostasis (WBC >100×10⁹/L): hydroxyurea, leukapheresis. DIC: FFP, cryoprecipitate, heparin in select cases |
| Nutrition | Neutropenic diet during chemotherapy; high-calorie, high-protein diet; parenteral nutrition if mucositis prevents oral intake |
| Diagnostic Summary | ≥20% blasts in BM + Auer rods + cytogenetic/molecular markers = AML |
11. ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)
| Domain | Details |
|---|---|
| Investigations | CBC, peripheral smear (lymphoblasts), bone marrow biopsy (>20–25% lymphoblasts), immunophenotyping (B-cell vs T-cell), cytogenetics (Philadelphia chromosome t(9;22) — worst prognosis), LP (CNS involvement), LDH (prognostic) |
| Findings | Lymphoblasts, anemia, thrombocytopenia, lymphadenopathy, hepatosplenomegaly, mediastinal mass (T-cell ALL) |
| Differential Dx | AML, NHL, viral lymphocytosis (EBV, CMV), CLL |
| Treatment | Induction: vincristine + dexamethasone + L-asparaginase ± daunorubicin. Ph+ ALL: TKI (imatinib/dasatinib) added. Consolidation + maintenance (2–3 years). CNS prophylaxis: intrathecal methotrexate ± cranial radiotherapy. Blinatumomab/inotuzumab (relapsed/refractory). CAR-T cell therapy |
| Contraindications | L-asparaginase contraindicated in pancreatitis; cranial RT avoided in young children |
| Blood Transfusion | As per AML; platelet transfusion threshold same; irradiated blood products mandatory |
| Blood Products | Irradiated, leukoreduced pRBC and platelets; FFP for coagulopathy |
| OPD Management | Maintenance therapy (6-mercaptopurine + methotrexate), PCP prophylaxis (co-trimoxazole), regular bone marrow monitoring |
| ICU Considerations | TLS, neutropenic sepsis, CNS leukemia, superior vena cava syndrome (mediastinal mass), typhlitis (neutropenic enterocolitis) |
| Nutrition | High-calorie, high-protein; parenteral nutrition if needed; avoid grapefruit (interacts with some chemotherapy) |
| Diagnostic Summary | Lymphoblasts in BM + immunophenotyping + cytogenetics (Philadelphia chromosome status) = ALL |
12. CHRONIC MYELOID LEUKEMIA (CML)
| Domain | Details |
|---|---|
| Investigations | CBC (↑ WBC with full myeloid spectrum, basophilia, eosinophilia), peripheral smear, Philadelphia chromosome (BCR-ABL1 t(9;22)) by FISH or PCR, bone marrow biopsy, LDH, uric acid |
| Findings | WBC 50–500×10⁹/L, low LAP score, basophilia, splenomegaly, BCR-ABL1 positive |
| Differential Dx | Leukemoid reaction, ET, primary myelofibrosis |
| Treatment | Tyrosine kinase inhibitors (TKI): imatinib (1st-line), dasatinib or nilotinib (2nd-generation), ponatinib (T315I mutation). TKI discontinuation possible after sustained deep molecular response (DMR). HSCT for blast phase |
| Contraindications | Nilotinib contraindicated in QTc prolongation, pancreatitis; dasatinib causes pleural effusion — avoid in lung disease |
| Blood Transfusion | Rarely needed in chronic phase; required in blast crisis |
| Blood Products | pRBC, platelets in blast phase |
| OPD Management | Monthly CBC initially; BCR-ABL1 PCR every 3 months; monitor TKI toxicity (QTc, LFTs, fluid retention) |
| ICU Considerations | Blast crisis: treat as AML/ALL; leukostasis: hydroxyurea emergency; TLS prophylaxis |
| Nutrition | Avoid grapefruit with nilotinib (CYP3A4 inhibitor); avoid high-purine diet |
| Diagnostic Summary | ↑ WBC + basophilia + BCR-ABL1 (Philadelphia chromosome) = CML |
13. CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
| Domain | Details |
|---|---|
| Investigations | CBC (lymphocytosis >5×10⁹/L), peripheral smear (smudge cells, small mature lymphocytes), immunophenotyping (CD5+, CD19+, CD23+, CD20 dim), FISH (del 17p, del 11q, del 13q, trisomy 12), IGHV mutation status |
| Findings | Lymphocytosis, smudge cells, lymphadenopathy, Rai/Binet staging |
| Differential Dx | NHL, SLL, hairy cell leukemia, reactive lymphocytosis, prolymphocytic leukemia |
| Treatment | Early/low-risk: watch and wait. Active disease: BTK inhibitor (ibrutinib, acalabrutinib); venetoclax + obinutuzumab; FCR (fludarabine, cyclophosphamide, rituximab) in young fit IGHV-mutated. 17p del: venetoclax or ibrutinib preferred |
| Contraindications | Fludarabine contraindicated in 17p deletion (poor response); ibrutinib with warfarin (bleeding risk) |
| Blood Transfusion | Irradiated blood products (risk of transfusion-associated GvHD due to immunosuppression); pRBC for AIHA-induced severe anemia |
| Blood Products | Irradiated pRBC, IVIG for recurrent infections (hypogammaglobulinemia) |
| OPD Management | Monitor for transformation (Richter), AIHA (DAT), infection prophylaxis, vaccinations |
| ICU Considerations | Richter transformation (aggressive DLBCL), pneumonia (Pneumocystis, fungal), sepsis |
| Nutrition | High-protein diet; adequate caloric intake; hydration |
| Diagnostic Summary | Lymphocytosis >5×10⁹/L × >3 months + CD5+/CD19+/CD23+ immunophenotype = CLL |
14. HODGKIN LYMPHOMA (HL)
| Domain | Details |
|---|---|
| Investigations | CBC, LDH, ESR, CXR (mediastinal widening), CT chest/abdomen/pelvis, PET-CT scan, lymph node excisional biopsy (Reed-Sternberg cells — CD30+, CD15+, CD45-), bone marrow biopsy, Ann Arbor staging |
| Findings | Reed-Sternberg cells on biopsy, B symptoms (fever, night sweats, >10% weight loss), lymphadenopathy (painless, rubbery), mediastinal mass |
| Differential Dx | NHL, sarcoidosis, infectious mononucleosis, metastatic carcinoma, CLL |
| Treatment | Early stage (I–IIA): ABVD × 2–4 cycles + radiation. Advanced (III–IV): ABVD × 6 cycles or escalated BEACOPP. Relapsed/refractory: brentuximab vedotin + nivolumab + auto-HSCT |
| Contraindications | Bleomycin (in ABVD) contraindicated in pulmonary fibrosis; radiation contraindicated in pregnancy |
| Blood Transfusion | Chemotherapy-induced anemia: pRBC if Hb <8 g/dL; ESA as alternative |
| Blood Products | pRBC (irradiated if post-HSCT or on fludarabine analogue), platelets |
| OPD Management | PET-CT response assessment at mid-treatment and end of treatment, long-term cardiac/pulmonary monitoring (late effects of radiation) |
| ICU Considerations | SVC syndrome from mediastinal mass: urgent steroids ± radiation; febrile neutropenia; tumor lysis |
| Nutrition | High-protein, high-calorie diet; anti-emetics with chemotherapy; antiemetic regimen |
| Diagnostic Summary | Painless lymphadenopathy + Reed-Sternberg cells (CD30+/CD15+) + PET-CT staging = HL |
15. NON-HODGKIN LYMPHOMA (NHL)
| Domain | Details |
|---|---|
| Investigations | CBC, LDH (prognostic — IPI score), β2-microglobulin, CT/PET-CT, excisional lymph node biopsy with immunohistochemistry (CD20+ B-cell DLBCL; CD3+ T-cell), BM biopsy, FISH (BCL2, BCL6, MYC for double-hit lymphoma) |
| Findings | Lymphadenopathy, organomegaly, elevated LDH, B symptoms in aggressive subtypes |
| Differential Dx | HL, leukemia, reactive lymphadenopathy, carcinoma metastasis |
| Treatment | DLBCL (aggressive): R-CHOP (rituximab + cyclophosphamide + doxorubicin + vincristine + prednisolone) × 6 cycles. Follicular (indolent): watch and wait or R-bendamustine. Mantle cell: ibrutinib, bortezomib. Relapsed: CAR-T therapy (axicabtagene, tisagenlecleucel) |
| Contraindications | Anthracyclines (doxorubicin) contraindicated in EF <40%; rituximab requires HBV screening (risk of reactivation) |
| Blood Transfusion | pRBC for anemia; platelet transfusion for thrombocytopenia |
| Blood Products | pRBC (irradiated if on purine analogue therapy), platelets, IVIG |
| OPD Management | Response assessment by PET-CT, relapse monitoring, late cardiotoxicity screening |
| ICU Considerations | TLS (high-risk in high-grade NHL), CNS involvement (prophylactic intrathecal MTX), febrile neutropenia |
| Nutrition | High-protein, high-calorie; antiemetic support; parenteral nutrition if oral intake compromised |
| Diagnostic Summary | Lymphadenopathy + LDH ↑ + lymph node biopsy with immunohistochemistry + CT/PET staging = NHL |
16. HEMOPHILIA A & B
| Domain | Details |
|---|---|
| Investigations | CBC, coagulation screen (↑ aPTT, normal PT), factor VIII assay (Hemophilia A), factor IX assay (Hemophilia B), mixing studies (correct with normal plasma — confirms deficiency, not inhibitor), inhibitor screening (Bethesda assay) |
| Findings | Prolonged aPTT, low factor VIII or IX, spontaneous hemarthroses, deep muscle hematomas |
| Differential Dx | vWD (Type 3), liver disease, factor XI deficiency, heparin therapy |
| Treatment | Hemophilia A: recombinant FVIII concentrate (on-demand or prophylaxis); emicizumab (bispecific antibody, SC). Hemophilia B: recombinant FIX; eptacog beta. Mild Hemophilia A: DDAVP (desmopressin). Inhibitors: bypassing agents (aPCC, recombinant FVIIa); immune tolerance induction |
| Contraindications | NSAIDs and aspirin contraindicated (increase bleeding); IM injections contraindicated; femoral venipuncture should be avoided |
| Blood Transfusion | FFP (if specific factor concentrate unavailable — less effective); cryoprecipitate for FVIII |
| Blood Products | Recombinant or plasma-derived FVIII/FIX concentrates; cryoprecipitate (FVIII, fibrinogen, vWF); FFP; aPCC (FEIBA); recombinant FVIIa (NovoSeven) |
| OPD Management | Prophylactic factor replacement (2–3× weekly or emicizumab SC weekly/biweekly/monthly); physiotherapy for joints; dental hygiene; inhibitor screening annually |
| ICU Considerations | Life-threatening bleeds (intracranial, airway, retroperitoneal): immediate high-dose factor replacement; neurosurgical consultation; target 80–100% factor level |
| Nutrition | Adequate vitamin K intake; maintain healthy weight (reduces joint stress); avoid supplements that affect coagulation (fish oil, vitamin E in high doses) |
| Diagnostic Summary | Prolonged aPTT + normal PT + low factor VIII (hemophilia A) or IX (hemophilia B) + X-linked inheritance = hemophilia |
17. DISSEMINATED INTRAVASCULAR COAGULATION (DIC)
| Domain | Details |
|---|---|
| Investigations | CBC (thrombocytopenia), PT ↑, aPTT ↑, fibrinogen ↓, D-dimer ↑↑, FDP ↑, peripheral smear (schistocytes), ISTH DIC score |
| Findings | Simultaneous thrombosis and hemorrhage, MODS, ISTH score ≥5 = overt DIC |
| Differential Dx | TTP, HUS, liver disease, severe vitamin K deficiency, massive transfusion |
| Treatment | Treat underlying cause (sepsis, obstetric emergency, malignancy). Supportive: FFP (1:1:1 ratio in massive hemorrhage), cryoprecipitate (fibrinogen <1.5 g/L), platelet transfusion (<50×10⁹/L with bleeding). Heparin only in thrombosis-dominant DIC (e.g., Trousseau syndrome) |
| Contraindications | Aminocaproic acid (antifibrinolytic) generally contraindicated (may worsen thrombosis); heparin contraindicated in hemorrhage-dominant DIC |
| Blood Transfusion | Massive transfusion protocol: pRBC:FFP:platelets = 1:1:1 |
| Blood Products | FFP, cryoprecipitate, fibrinogen concentrate, platelet concentrates, pRBC, TXA (tranexamic acid) in trauma-DIC |
| OPD Management | Manage underlying disease; monitor coagulation parameters |
| ICU Considerations | Central to ICU care: MODS management, vasopressors, ventilation, renal replacement therapy; ISTH DIC score daily; goal-directed coagulation correction |
| Nutrition | Enteral nutrition preferred in ICU; parenteral if gut non-functional; adequate protein |
| Diagnostic Summary | Clinical context (sepsis/trauma/malignancy) + ↓ fibrinogen + ↑ D-dimer + ↑ PT/aPTT + ↓ platelets + schistocytes = DIC (ISTH score ≥5) |
18. IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP)
| Domain | Details |
|---|---|
| Investigations | CBC (isolated thrombocytopenia, normal Hb/WBC), peripheral smear (large platelets, no schistocytes), bone marrow (increased megakaryocytes — only if diagnosis uncertain), ANA, APLA screen, H. pylori testing, HIV/HCV serology |
| Findings | Platelet count <100×10⁹/L (often <30×10⁹/L), petechiae, purpura, mucosal bleeding; no splenomegaly |
| Differential Dx | TTP, drug-induced thrombocytopenia (heparin — HIT), SLE, hypersplenism, MDS, sepsis-induced |
| Treatment | Observation if platelets >30×10⁹/L and asymptomatic. Active bleeding or <30×10⁹/L: prednisolone 1 mg/kg/day; IVIG 1 g/kg for rapid response. Second-line: rituximab, thrombopoietin receptor agonists (eltrombopag, romiplostim), splenectomy. H. pylori eradication if positive |
| Contraindications | Avoid NSAIDs and antiplatelet drugs; splenectomy requires vaccination (pneumococcal, meningococcal, Hib); avoid platelet transfusion routinely (destroyed immediately) |
| Blood Transfusion | Platelet transfusion only for life-threatening bleeding (intracranial hemorrhage) or emergency surgery; pRBC if co-existing anemia |
| Blood Products | Platelet concentrates (emergency only), IVIG, anti-D immunoglobulin (Rh-positive patients) |
| OPD Management | Monitor platelet counts; gradual steroid taper; H. pylori eradication; eltrombopag for chronic ITP |
| ICU Considerations | Intracranial hemorrhage: emergency IVIG + high-dose steroids + platelet transfusion; platelet target >50–100×10⁹/L for procedures |
| Nutrition | Avoid fish oil, vitamin E (affect platelet function); adequate nutrition |
| Diagnostic Summary | Isolated thrombocytopenia + large platelets + normal Hb/WBC + bone marrow megakaryocytes ↑ + exclusion of secondary causes = ITP |
19. THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP)
| Domain | Details |
|---|---|
| Investigations | CBC (microangiopathic hemolytic anemia + thrombocytopenia), peripheral smear (schistocytes — key), ADAMTS13 activity (<10% confirms TTP), LDH ↑↑, haptoglobin ↓, Coombs test (negative), renal function, troponin, bilirubin |
| Findings | Classic pentad (rare to see all 5): MAHA + thrombocytopenia + neurological symptoms + renal failure + fever |
| Differential Dx | HUS, DIC, HELLP syndrome, APS, malignant hypertension, drug-induced TMA (quinine, cyclosporine) |
| Treatment | Medical emergency: URGENT plasma exchange (PEX) — fresh frozen plasma (FFP) 1–1.5× plasma volume daily. Caplacizumab (anti-vWF nanobody) + PEX + immunosuppression (steroids ± rituximab) dramatically reduces mortality. ADAMTS13 replacement in congenital TTP |
| Contraindications | Platelet transfusion is CONTRAINDICATED (worsens thrombosis — "fuel on the fire"); do not delay PEX waiting for ADAMTS13 result |
| Blood Transfusion | pRBC only if Hb critically low; NO platelet transfusion |
| Blood Products | FFP (for plasma exchange), NO platelets |
| OPD Management | ADAMTS13 monitoring post-remission; rituximab for relapse prevention; caplacizumab maintenance |
| ICU Considerations | Neurological deterioration, cardiac troponin elevation (poor prognosis), renal failure: daily PEX; ICU monitoring; avoid platelet-activating procedures |
| Nutrition | ICU-level nutritional support; enteral feeding preferred |
| Diagnostic Summary | MAHA + thrombocytopenia + schistocytes + ADAMTS13 <10% = TTP (treat before ADAMTS13 returns if clinically suspected) |
20. MULTIPLE MYELOMA (MM)
| Domain | Details |
|---|---|
| Investigations | CBC (normocytic anemia, Rouleaux formation), serum protein electrophoresis (M-band/spike), serum free light chains (kappa/lambda ratio), 24-hour urine protein electrophoresis (Bence Jones protein), β2-microglobulin, LDH, calcium, creatinine, skeletal survey/whole-body low-dose CT/PET-CT, bone marrow biopsy (≥10% clonal plasma cells), FISH (del 17p, t(4;14), t(14;16) — high-risk cytogenetics) |
| Findings | CRAB criteria: hyperCalcemia, Renal failure, Anemia, Bone lesions (lytic lesions). Rouleaux on smear, elevated ESR, low normal gap |
| Differential Dx | MGUS, Waldenstrom macroglobulinemia, primary amyloidosis, metastatic carcinoma, POEMS syndrome |
| Treatment | Transplant-eligible: VRd (bortezomib + lenalidomide + dexamethasone) induction → auto-HSCT → lenalidomide maintenance. Transplant-ineligible: VRd or DRd (daratumumab + Rd). Relapsed/refractory: daratumumab, carfilzomib, pomalidomide, elotuzumab, belantamab, CAR-T (idecabtagene vicleucel) |
| Contraindications | Thalidomide/lenalidomide require VTE prophylaxis; bortezomib requires HSV prophylaxis (aciclovir); bisphosphonates (zoledronic acid) require dental assessment first (risk of osteonecrosis of jaw) |
| Blood Transfusion | pRBC for symptomatic anemia (Hb <8 g/dL); ESAs if EPO-driven anemia; no ESAs if CRAB present |
| Blood Products | pRBC (irradiated if on immunomodulatory agents); IVIG for recurrent infections (hypogammaglobulinemia); platelet transfusion for thrombocytopenia |
| OPD Management | Regular serum free light chain and M-protein monitoring, renal function, calcium, bisphosphonate infusions, VTE prophylaxis, vaccination |
| ICU Considerations | Hyperviscosity syndrome (confusion, bleeding, visual changes): emergency plasmapheresis. Hypercalcemic crisis: IV fluids, IV bisphosphonate, calcitonin, steroids. Spinal cord compression: emergency MRI + dexamethasone ± radiation/surgery. Renal failure: hydration, alkalinization, bortezomib-based regimen (most renally safe) |
| Nutrition | High-protein (caution in renal failure), adequate hydration (critical for myeloma kidney), restrict phosphate if renal failure; calcium restriction if hypercalcemia |
| Diagnostic Summary | CRAB criteria + ≥10% clonal plasma cells in BM + M-protein on electrophoresis + FISH cytogenetics = Multiple Myeloma |
🩸 BLOOD PRODUCT SUMMARY TABLE
| Product | Indication | Special Notes |
|---|---|---|
| Packed RBCs (pRBC) | Anemia (Hb <7–8 g/dL), symptomatic anemia | 1 unit raises Hb ~1 g/dL; leukoreduced to prevent febrile reactions |
| Fresh Frozen Plasma (FFP) | DIC, TTP (plasma exchange), liver disease, factor deficiencies | Contains all coagulation factors; must be ABO compatible |
| Cryoprecipitate | Hemophilia A (no concentrate available), DIC (fibrinogen <1.5), vWD | Contains FVIII, vWF, fibrinogen, FXIII |
| Platelet Concentrates | Thrombocytopenia with bleeding or <10×10⁹/L prophylactic | Contraindicated in TTP, HIT; irradiate for immunocompromised |
| Albumin | Hypoalbuminemia, hepatic cirrhosis, SBP, large-volume paracentesis | 4–5% for volume expansion; 20–25% for hypoalbuminemia |
| IVIG | ITP, AIHA, hypogammaglobulinemia (CLL), Kawasaki | Monitor renal function; slow infusion to prevent reactions |
| Factor Concentrates | Hemophilia A (FVIII), Hemophilia B (FIX) | Recombinant preferred (lower infection risk) |
| Anti-D Immunoglobulin | Rh-negative mothers, ITP (Rh-positive patients) | Prevents Rh sensitization |
| Granulocyte Transfusion | Severe neutropenia with refractory infection | Short shelf-life; irradiated; rarely used |
🏥 ICU vs OPD MANAGEMENT PRINCIPLES
| Setting | Key Principles |
|---|---|
| ICU | Restrictive transfusion strategy (Hb threshold 7 g/dL unless cardiac disease → 8 g/dL); treat the cause, not just numbers; daily CBC; DIC scoring; neutropenic precautions; hemodynamic monitoring; avoid unnecessary platelet transfusions |
| OPD | Monitor CBC at defined intervals; oral/SC treatments preferred; educate patients on warning signs; vaccination for immunocompromised; manage comorbidities; follow disease-specific response criteria (CR/PR/SD/PD) |
🥗 NUTRITIONAL GUIDANCE IN BLOOD DISORDERS
| Condition | Key Nutritional Advice |
|---|---|
| IDA | Iron-rich foods + vitamin C; avoid tea/coffee with meals |
| Megaloblastic anemia | Folate (leafy greens) + B12 (meat, dairy) |
| Thalassemia/Hemochromatosis | Low-iron diet; avoid alcohol |
| Neutropenic patients | Neutropenic diet (no raw/undercooked food) |
| Myeloma + renal failure | Low-phosphate, controlled protein, high hydration |
| Lymphoma/Leukemia on chemo | High-calorie, high-protein; anti-emetic support; TPN if mucositis |
Key References:
- Goldman-Cecil Medicine (International Edition, 2-Volume Set) — Blood chapters
- Henry's Clinical Diagnosis and Management by Laboratory Methods
- Tietz Textbook of Laboratory Medicine, 7th Edition
- Katzung's Basic and Clinical Pharmacology, 16th Edition
- The Washington Manual of Medical Therapeutics
- ISTH Guidelines for DIC; ASH Guidelines for ITP, AML, TTP, Hemophilia
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