I am studying pathology for NEET PG 2026 MCQ exam. Current topic is thrombotic thrombocytopenic purpura. So I want you to give a summary of this topic covering almost 100% of the questions that can be asked for this exam.
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I now have comprehensive data from Robbins, Harrison's, Quick Compendium, Washington Manual, Rosen's Emergency Medicine, and Comprehensive Clinical Nephrology. I have everything needed for a thorough NEET PG-focused summary. Let me compile this now.
Thrombotic Thrombocytopenic Purpura (TTP) — NEET PG 2026 Master Summary
1. DEFINITION & CLASSIFICATION
TTP is a thrombotic microangiopathy (TMA) characterised by systemic platelet-rich microthrombi causing:
- Microangiopathic hemolytic anemia (MAHA)
- Consumptive thrombocytopenia
- Organ ischemia (brain, kidney, heart)
Classification:
| Type | Mechanism |
|---|---|
| Congenital/Hereditary TTP (Upshaw-Schulman syndrome) | Homozygous or compound heterozygous ADAMTS13 gene mutation → absent/severely reduced enzyme |
| Acquired (Idiopathic/Immune) TTP | IgG autoantibodies against ADAMTS13 (accounts for 60–90% of acute cases) |
| Secondary TTP | Triggered by drugs, HIV, pregnancy, SLE, malignancy — NOT strongly associated with ADAMTS13 deficiency |
2. PATHOGENESIS — THE ADAMTS13 STORY ⭐ (Most Important)
What is ADAMTS13?
- A Disintegrin-like And Metalloproteinase with Thrombospondin type-1 motif, member 13
- Also called: von Willebrand factor (vWF) cleaving protease
- Synthesised mainly in the liver (also in renal podocytes, tubular cells, endothelial cells, platelets)
- Half-life: 1–2 days in plasma
Normal Function:
- vWF is synthesised in Weibel-Palade bodies of endothelial cells and megakaryocytes
- Released as ultra-large (UL) vWF multimers
- ADAMTS13 cleaves these UL-vWF multimers → normal-sized, less reactive vWF forms
In TTP:
- ADAMTS13 is deficient or absent (activity ≤5–10%)
- UL-vWF multimers accumulate in plasma
- Under shear stress in arterioles/capillaries, UL-vWF binds platelets via GPIb-IX-V
- Platelet-rich hyaline microthrombi form throughout the microcirculation
- Red cells fragment against these thrombi → schistocytes
- Platelets are consumed → thrombocytopenia
- Organ ischemia (brain, kidney, heart, adrenals)
Key concept: The primary defect is in platelet activation (NOT coagulation cascade), so PT and aPTT are normal — this distinguishes TTP from DIC.
3. CLASSIC CLINICAL PENTAD ⭐⭐
"FAT RN" (Fever, Anemia [hemolytic], Thrombocytopenia, Renal dysfunction, Neurologic symptoms)
| Feature | Detail |
|---|---|
| Thrombocytopenia | Platelets commonly <20,000/µL |
| Microangiopathic hemolytic anemia | Schistocytes on smear, ↑LDH, ↑unconjugated bilirubin, ↓haptoglobin |
| Neurologic symptoms | Fluctuating: confusion, headache, TIA, seizures, stroke (most characteristic) |
| Renal dysfunction | Milder than HUS; creatinine typically <2.0 mg/dL at presentation |
| Fever | Low-grade |
Exam trap: The complete pentad is present in <30% of patients. Most patients present with only thrombocytopenia + MAHA. Do not wait for the full pentad to treat.
4. EPIDEMIOLOGY
- More common in women than men (~2:1)
- More common in HIV-infected patients and pregnant women
- Congenital TTP: 5–10 cases/million/year; Acquired TTP: ≤1 case/million/year
- Acquired TTP: ~90% of first episodes occur in adults
5. LABORATORY FINDINGS ⭐⭐
| Test | Result in TTP |
|---|---|
| Peripheral smear | Schistocytes (>3–5 per HPF), helmet cells, polychromasia |
| Platelet count | <20,000/µL (severe thrombocytopenia) |
| LDH | Very elevated, often >1,000 IU/dL |
| Unconjugated bilirubin | Elevated |
| Haptoglobin | Low or undetectable |
| Reticulocyte count | Elevated (>2.5%) |
| PT/aPTT | Normal (key feature — unlike DIC) |
| Creatinine | Typically <2.0 mg/dL at onset |
| DAT (Coombs test) | Negative (non-immune hemolysis) |
| ADAMTS13 activity | ≤5–10% (diagnostic of primary TTP) |
| ADAMTS13 inhibitor | Present in acquired TTP |
| Fibrinogen | Normal (unlike DIC where it is consumed) |
6. PLASMIC SCORE ⭐ (For Predicting Probability of Acquired TTP)
PLASMIC = Platelets, Lysis, Active cancer, Solid organ/stem cell transplant, MCV, INR, Creatinine
| Criterion | Score |
|---|---|
| Platelet count <30 × 10⁹/L | +1 |
| Evidence of hemolysis (reticulocytes >2.5%, or haptoglobin undetectable, or indirect bili >2 mg/dL) | +1 |
| No active cancer / treated for cancer in past year | +1 |
| No history of solid organ or stem cell transplant | +1 |
| MCV <90 fL | +1 |
| INR <1.5 | +1 |
| Creatinine <2.0 mg/dL | +1 |
Interpretation:
- 0–4: Low risk → consider alternative diagnosis
- 5: Intermediate risk → consult hematology, consider plasma exchange
- 6–7: High risk → immediate plasma exchange
7. PATHOLOGY (Morphology) ⭐
- Hyaline microthrombi in arterioles and capillaries of multiple organs (brain, kidney, heart, adrenals)
- Thrombi composed predominantly of platelets and vWF (NOT fibrin-rich like DIC thrombi)
- No vessel wall inflammation (unlike vasculitis)
- Onion-skin intimal thickening of arterioles in some vessels
- Organs most affected: brain, kidney, adrenals, pancreas, heart
8. TTP vs HUS — KEY DIFFERENCES ⭐⭐⭐
| Feature | TTP | HUS (Typical) |
|---|---|---|
| ADAMTS13 activity | ≤5–10% (severely low) | Normal (30–100%) |
| Cause | ADAMTS13 deficiency (autoantibody or genetic) | Shiga toxin (E. coli O157:H7) or complement dysregulation |
| Predominant feature | Neurologic symptoms | Renal failure (AKI) |
| Renal dysfunction | Mild (creatinine usually <2) | Severe — major feature |
| Age | Adults (mainly) | Children (mainly) |
| Diarrhea prodrome | Absent | Present (bloody diarrhea in typical HUS) |
| ADAMTS13 inhibitor | Present (acquired) | Absent |
| Treatment | Plasma exchange (mandatory) | Supportive; eculizumab for aHUS |
| Platelet transfusion | Contraindicated | Less strictly contraindicated |
9. TTP vs DIC — CRITICAL DIFFERENCES ⭐⭐
| Feature | TTP | DIC |
|---|---|---|
| PT/aPTT | Normal | Prolonged |
| Fibrinogen | Normal | Low (consumed) |
| D-dimer/FDPs | Normal/mildly elevated | Very elevated |
| Pathogenesis | Platelet activation (primary) | Coagulation cascade activation |
| Thrombus type | Platelet-rich (vWF-rich) | Fibrin-rich |
| Treatment | Plasma exchange | Treat underlying cause; FFP/cryo |
10. TREATMENT ⭐⭐⭐
First-Line: Therapeutic Plasma Exchange (TPE/PEX) ⭐
- Mainstay of treatment — do NOT wait for ADAMTS13 results before starting
- Replaces 1.0–1.5 plasma volumes daily
- Replacement fluid: Thawed plasma / FFP (provides functional ADAMTS13)
- Cryopoor plasma (depleted of UL-vWF) used as alternative by some centres
- Before TTP — mortality was ~90%; with TPE, remission achieved in >80% of patients
- Continue until platelet count normalises AND signs of hemolysis resolved for ≥2 days
If TPE is unavailable or delayed:
- Give FFP infusion (10–15 mL/kg) as a bridge to TPE
Adjunct Therapies:
| Agent | Role |
|---|---|
| Glucocorticoids (prednisone 1 mg/kg/day or methylprednisolone IV) | Adjunct to suppress autoantibody production |
| Rituximab (anti-CD20) | Reduces relapse risk; added to initial therapy; 375 mg/m² IV |
| Caplacizumab | Anti-vWF nanobody (humanized single variable domain immunoglobulin); blocks vWF–GPIb-IX-V interaction → prevents platelet aggregation; approved for acquired TTP; used with TPE + immunosuppression; HERCULES trial showed ↓mortality, faster platelet normalisation, fewer TPE days, ↓30-day relapse |
| Splenectomy | Refractory/last resort cases |
For Congenital (Hereditary) TTP (Upshaw-Schulman):
- Prophylactic plasma transfusions to prevent acute episodes
- Recombinant ADAMTS13 (under investigation — phase III trial)
CONTRAINDICATED:
- Platelet transfusion — relatively contraindicated (may "fuel the fire" by worsening microvascular occlusions); only used if severe bleeding or invasive procedure needed (platelets <10,000/µL)
11. PROGNOSIS AND RELAPSE
- Remission in ~90% with treatment
- Relapse rate: 25–45% within 30 days of initial remission; 12–40% have late relapses
- Relapses more common with persistently low ADAMTS13 (<10%) at presentation
- Persistently low ADAMTS13 → increased risk of stroke
- Rituximab reduces relapse risk
12. SECONDARY / DRUG-INDUCED TTP ⭐
Drugs associated with TTP:
- Antibody-mediated (severe ADAMTS13 deficiency): Ticlopidine (80–90%), clopidogrel (few cases)
- Endothelial toxicity (NOT ADAMTS13-mediated): Cyclosporine, tacrolimus, gemcitabine, mitomycin C
- Others: HIV, SLE, lymphoid neoplasms, bone marrow transplantation, radiation, pregnancy
Management of drug-induced TTP: Discontinue the offending drug (usually leads to recovery in endothelial toxicity type)
13. PREGNANCY AND TTP
- TTP in pregnancy most often in 2nd/3rd trimester (unlike HELLP which is ≥20 weeks with different features)
- Pregnancy is a triggering event even in genetic ADAMTS13 deficiency
- Distinguish TTP from HELLP:
- HELLP: preceded by HTN, proteinuria, severe liver injury → delivery is treatment
- TTP: renal failure + severe non-immune hemolytic anemia → plasma exchange is treatment; delivery does NOT alter TTP course
14. HIGH-YIELD FACTS FOR MCQs ⭐⭐⭐
| Question Theme | Answer |
|---|---|
| Enzyme deficient in TTP | ADAMTS13 (vWF-cleaving metalloprotease) |
| Where is ADAMTS13 made? | Primarily liver |
| Storage site of vWF in endothelial cells | Weibel-Palade bodies |
| Type of anemia in TTP | Microangiopathic hemolytic anemia (MAHA) |
| Coombs test in TTP | Negative (non-immune) |
| PT/aPTT in TTP | Normal |
| Most characteristic cells on smear | Schistocytes (helmet cells) |
| Inherited TTP (congenital) name | Upshaw-Schulman syndrome |
| Treatment of choice (acute TTP) | Therapeutic plasma exchange (TPE) |
| Replacement fluid used in TPE | FFP / Thawed plasma |
| Newer targeted drug for TTP | Caplacizumab (anti-vWF nanobody) |
| Why are platelets contraindicated? | Risk of worsening microvascular occlusion |
| Classic pentad is complete in what % | <30% of cases |
| Mortality before TPE was available | ~90% |
| Mortality/remission with TPE | >80% achieve remission |
| Key differentiator: TTP vs HUS | TTP → Neuro dominant, ADAMTS13 low; HUS → Renal dominant, ADAMTS13 normal |
| Key differentiator: TTP vs DIC | PT/aPTT normal in TTP, prolonged in DIC |
| Drug most associated with antibody-mediated TTP | Ticlopidine |
| PLASMIC score high risk cutoff | 6–7 points |
| Trial for caplacizumab | HERCULES trial |
| vWF receptor on platelets | GPIb-IX-V |
| Thrombus type in TTP | Hyaline (platelet-rich, vWF-rich), NOT fibrin-rich |
15. QUICK MNEMONICS
- Pentad: "FAT RN" — Fever, Anemia (hemolytic), Thrombocytopenia, Renal dysfunction, Neurologic deficits
- PLASMIC score — Platelets, Lysis, Active cancer, Stem cell/Solid organ transplant, MCV, INR, Creatinine
- TTP = Neuro; HUS = Renal (for the exam)
- ADAMTS13 ≤5–10% = TTP; ADAMTS13 normal = HUS
Sources: Robbins & Cotran Pathologic Basis of Disease (10e) — Quick Compendium of Clinical Pathology (5e) — Harrison's Principles of Internal Medicine (22e) — Washington Manual of Medical Therapeutics — Comprehensive Clinical Nephrology (7e) — Henry's Clinical Diagnosis & Management by Laboratory Methods
I want you to convert this info into a summary that is into an Anki card. It will be imported to AnkiDroid app. It should be in a single line. Front should be Thrombotic Thrombocytopenic Purpura, and the back should be the summary. It should be in a single line. The front part of the card should show the heading, back should be the summary. There should be proper spacing in the summary for easy readability. It should include only the most important points, and it should be a self-explanatory summary.
This summary should be in a text format so that I can copy and paste it to a text app and then put on Keydroid app, and it should be in a single line.
The summary should be in a single line and there should be proper spacing. It should also be in an import-ready format.
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Front[TAB]Back — all on one line, with <br> tags for line breaks inside the back field (since HTML is supported in Anki). Here is your import-ready card:Thrombotic Thrombocytopenic Purpura 🔬 DEFINITION: TMA caused by ADAMTS13 deficiency → UL-vWF accumulation → platelet-rich hyaline microthrombi in microcirculation → MAHA + thrombocytopenia + organ ischemia.<br><br>📌 TYPES: (1) Congenital = Upshaw-Schulman syndrome (ADAMTS13 gene mutation) | (2) Acquired/Immune = IgG autoAb against ADAMTS13 (60-90% of cases) | (3) Secondary = drugs/HIV/pregnancy (NOT ADAMTS13-mediated).<br><br>⭐ ADAMTS13: vWF-cleaving metalloprotease. Made in LIVER. Stored in Weibel-Palade bodies (vWF). Activity ≤5-10% = TTP.<br><br>🩺 PENTAD (FAT RN) — present in <30% cases: Fever | Anemia (MAHA) | Thrombocytopenia | Renal dysfunction | Neurologic deficits (fluctuating).<br><br>🔍 LABS: Schistocytes on smear (>3-5/HPF) | Platelets <20,000 | LDH >1000 IU/dL | ↑Unconjugated bili | ↓Haptoglobin | PT/aPTT NORMAL | Coombs NEGATIVE | Creatinine typically <2.0 mg/dL.<br><br>📊 PLASMIC SCORE (1pt each): Plt <30 | Hemolysis | No cancer | No transplant | MCV <90fL | INR <1.5 | Creatinine <2.0 → Score 0-4=Low | 5=Intermediate | 6-7=High risk → immediate plasma exchange.<br><br>💊 TREATMENT: (1) Plasma Exchange (TPE) = MAINSTAY — FFP/thawed plasma as replacement fluid | continue till plt normal + hemolysis resolved ≥2 days. (2) If TPE delayed → FFP infusion (10-15 mL/kg) as bridge. (3) Steroids (prednisone 1mg/kg) — adjunct. (4) Rituximab (anti-CD20) — reduces relapse. (5) Caplacizumab (anti-vWF nanobody) — HERCULES trial — blocks vWF-GPIb interaction → approved for acquired TTP. (6) Congenital TTP → prophylactic plasma transfusions.<br><br>🚫 CONTRAINDICATED: Platelet transfusion (worsens microvascular occlusion) — only if severe bleeding or plt <10,000.<br><br>⚡ TTP vs HUS: TTP = Neuro dominant + ADAMTS13 low + adults | HUS = Renal dominant + ADAMTS13 normal + children + E.coli O157:H7.<br><br>⚡ TTP vs DIC: PT/aPTT NORMAL in TTP | Prolonged in DIC | Fibrinogen normal in TTP | Low in DIC.<br><br>📈 PROGNOSIS: Pre-TPE mortality = 90% | With TPE remission >80% | Relapse 25-45% within 30 days | Rituximab reduces relapse.<br><br>💊 DRUGS: Ticlopidine (Ab-mediated, ADAMTS13 low 80-90%) | Cyclosporine/Gemcitabine/Mitomycin C (endothelial toxicity, ADAMTS13 normal) → stop drug.How to import into AnkiDroid:
- Copy everything between the backticks above (the single line starting with
Thrombotic...) - Paste into a
.txtfile - In AnkiDroid → Import → select the file → field separator: Tab → import
The bears and we have thrombotic thrombocytopenia covering almost 100% of pathology aspect of this topic that can be asked for NEAT PG exam. It should be in a text format so that I can copy and paste and it should be in a single line with proper spacing. The front part of the card should be TTP and the back part should be the summary.
Here is your Anki import-ready card focused on the pathology of TTP:
TTP 📌 DEFINITION: Thrombotic Microangiopathy (TMA) — systemic platelet-rich hyaline microthrombi in microcirculation → MAHA + thrombocytopenia + organ ischemia.<br><br>⭐ PATHOGENESIS: ADAMTS13 (vWF-cleaving metalloprotease, made in LIVER) deficiency → UL-vWF multimers accumulate → under shear stress, UL-vWF binds platelets via GPIb-IX-V → platelet-rich microthrombi → RBCs fragment against thrombi (schistocytes) → consumptive thrombocytopenia + organ ischemia. PRIMARY DEFECT = platelet activation, NOT coagulation cascade → PT/aPTT NORMAL.<br><br>🔬 MORPHOLOGY: Hyaline microthrombi (platelet + vWF rich, NOT fibrin-rich) in arterioles and capillaries of brain, kidney, heart, adrenals, pancreas | No vessel wall inflammation | Onion-skin intimal thickening in arterioles | Schistocytes + helmet cells on smear.<br><br>📌 TYPES: (1) Congenital = Upshaw-Schulman syndrome — homozygous/compound heterozygous ADAMTS13 mutation — episodic, onset adolescence | (2) Acquired/Immune = IgG autoAb against ADAMTS13 — 60-90% acute cases — autoAb disappear in remission | (3) Secondary = drugs/HIV/pregnancy/SLE/malignancy — NOT ADAMTS13-mediated.<br><br>🧬 ADAMTS13: Full name = A Disintegrin-like And Metalloproteinase with Thrombospondin type-1 motif 13 | Synthesised in LIVER (also renal podocytes, endothelial cells) | Plasma conc ~1 µg/mL | Half-life 1-2 days | vWF stored in Weibel-Palade bodies of endothelial cells and megakaryocytes.<br><br>🩺 PENTAD (FAT RN) — present in <30% cases: Fever | Anemia (MAHA) | Thrombocytopenia | Renal dysfunction (mild) | Neurologic deficits (fluctuating — most characteristic).<br><br>🔍 KEY LABS: Schistocytes >3-5/HPF | Plt <20,000/µL | LDH >1000 IU/dL | ↑Unconjugated bilirubin | ↓/absent haptoglobin | Reticulocytes >2.5% | PT/aPTT NORMAL | Fibrinogen NORMAL | Coombs (DAT) NEGATIVE | ADAMTS13 activity ≤5-10% | ADAMTS13 inhibitor present (acquired) | Creatinine typically <2.0 mg/dL.<br><br>⚡ TTP vs DIC: PT/aPTT = Normal in TTP vs Prolonged in DIC | Fibrinogen = Normal in TTP vs Low in DIC | Thrombus = Platelet-rich/hyaline in TTP vs Fibrin-rich in DIC | D-dimer = Normal/mild in TTP vs Very high in DIC.<br><br>⚡ TTP vs HUS: TTP = ADAMTS13 ≤5-10% + Neuro dominant + Adults + No diarrhea prodrome | HUS = ADAMTS13 Normal (30-100%) + Renal dominant (severe AKI) + Children + E.coli O157:H7 Shiga toxin + Diarrhea prodrome | aHUS = complement dysregulation (factor H/I/C3 mutations).<br><br>💊 TREATMENT: (1) PLASMA EXCHANGE (TPE) = MAINSTAY — replacement fluid FFP/thawed plasma (cryopoor plasma alternative) — 1.0-1.5 plasma volumes daily — continue till plt normal + hemolysis resolved ≥2 days — pre-TPE mortality 90%, post-TPE remission >80% | (2) If TPE delayed → FFP 10-15 mL/kg IV as bridge | (3) Steroids (prednisone 1mg/kg) adjunct | (4) Rituximab (anti-CD20, 375mg/m²) — reduces relapse | (5) Caplacizumab — anti-vWF nanobody — blocks vWF-GPIb-IX-V — HERCULES trial — faster plt recovery, fewer TPE days, ↓30-day relapse — approved for acquired TTP | (6) Congenital TTP → prophylactic plasma transfusions | (7) Refractory → splenectomy (last resort).<br><br>🚫 CONTRAINDICATED: Platelet transfusion — worsens microvascular occlusion — only if severe bleeding or plt <10,000/µL.<br><br>📌 DRUGS CAUSING TTP: Ab-mediated (ADAMTS13 low) = Ticlopidine (80-90%), Clopidogrel | Endothelial toxicity (ADAMTS13 normal) = Cyclosporine, Tacrolimus, Gemcitabine, Mitomycin C → stop drug.<br><br>📈 RELAPSE: 25-45% within 30 days | 12-40% late relapses | Risk ↑ if ADAMTS13 persistently <10% | Rituximab reduces relapse.To import: Copy the single line above (between the backticks) → save as
.txt file → AnkiDroid → Import → field separator: Tab.Also make 20 MCQs in the same above format covering most of the high-yield pathologic points from this topic which will help to answer MCQs for the NEET PG.
Here are 20 Anki-import-ready MCQ cards, one per line, tab-separated (Front = Question + Options, Back = Answer + Explanation):
Q1. A 35-year-old woman presents with confusion, fever, thrombocytopenia, and anemia. Peripheral smear shows fragmented RBCs. PT/aPTT are normal. Which enzyme is deficient in this condition? A) Factor VIII B) ADAMTS13 C) Protein C D) Thrombomodulin ✅ B) ADAMTS13 | TTP is caused by deficiency of ADAMTS13 (vWF-cleaving metalloprotease). Deficiency → UL-vWF accumulation → platelet-rich microthrombi. PT/aPTT are NORMAL (coagulation cascade is NOT activated — key distinguisher from DIC).Q2. ADAMTS13, deficient in TTP, is primarily synthesised in which organ? A) Spleen B) Kidney C) Liver D) Bone marrow ✅ C) Liver | ADAMTS13 is primarily synthesised in the LIVER. It is also expressed at lower levels in renal podocytes, tubular cells, vascular endothelial cells, and platelets. Half-life in plasma = 1-2 days.Q3. von Willebrand factor (vWF) is stored in which organelle of endothelial cells? A) Golgi apparatus B) Weibel-Palade bodies C) Lysosomes D) Endoplasmic reticulum ✅ B) Weibel-Palade bodies | vWF is stored in Weibel-Palade bodies of endothelial cells and in alpha-granules of megakaryocytes/platelets. On stimulation, UL-vWF multimers are released and must be cleaved by ADAMTS13 to prevent thrombosis.Q4. A child presents with bloody diarrhea followed by acute renal failure and thrombocytopenia. ADAMTS13 activity is normal. What is the most likely diagnosis? A) TTP B) DIC C) Typical HUS D) ITP ✅ C) Typical HUS | ADAMTS13 is NORMAL in HUS (30-100%). Typical HUS = E.coli O157:H7 Shiga toxin → endothelial damage → RENAL dominant disease + children + diarrhea prodrome. TTP = adults + Neuro dominant + ADAMTS13 ≤5-10%.Q5. Which of the following best distinguishes TTP from DIC? A) Presence of thrombocytopenia B) Normal PT/aPTT with normal fibrinogen in TTP C) Elevated LDH D) Presence of schistocytes ✅ B) Normal PT/aPTT with normal fibrinogen in TTP | In TTP, primary defect = platelet activation (NOT coagulation cascade) → PT/aPTT and fibrinogen are NORMAL. In DIC, coagulation factors are consumed → PT/aPTT prolonged + fibrinogen LOW + D-dimer very high.Q6. What type of microthrombi are characteristically seen in TTP? A) Fibrin-rich thrombi B) Red thrombi C) Hyaline (platelet + vWF-rich) thrombi D) Mixed platelet-fibrin thrombi ✅ C) Hyaline (platelet + vWF-rich) thrombi | TTP thrombi are HYALINE — composed predominantly of platelets and vWF, NOT fibrin. Fibrin-rich thrombi = DIC. Absence of vessel wall inflammation distinguishes TTP from vasculitis.Q7. The inherited form of TTP is known as: A) Bernard-Soulier syndrome B) Upshaw-Schulman syndrome C) Kasabach-Merritt syndrome D) Wiskott-Aldrich syndrome ✅ B) Upshaw-Schulman syndrome | Congenital/hereditary TTP = Upshaw-Schulman syndrome — caused by homozygous or compound heterozygous mutations in ADAMTS13 gene — autosomal recessive — episodic relapses — onset often delayed to adolescence — treated with prophylactic plasma transfusions.Q8. The classic pentad of TTP includes all EXCEPT: A) Fever B) Microangiopathic hemolytic anemia C) Neurologic deficits D) Elevated PT/aPTT ✅ D) Elevated PT/aPTT | The classic pentad (FAT RN) = Fever, Anemia (MAHA), Thrombocytopenia, Renal dysfunction, Neurologic deficits. PT/aPTT are NORMAL in TTP — this is a KEY high-yield point. The full pentad is present in <30% of cases.Q9. A peripheral blood smear in TTP most characteristically shows: A) Hypersegmented neutrophils B) Target cells C) Schistocytes (helmet cells) D) Spherocytes ✅ C) Schistocytes (helmet cells) | Schistocytes = fragmented RBCs that form when RBCs are sheared against platelet-vWF microthrombi in the microcirculation. >3-5 schistocytes per HPF is significant. This is the hallmark of MAHA (microangiopathic hemolytic anemia).Q10. Direct Antiglobulin Test (Coombs test) in TTP is: A) Strongly positive B) Weakly positive C) Negative D) Variable ✅ C) Negative | TTP causes NON-IMMUNE hemolysis (mechanical fragmentation of RBCs against microthrombi) → Coombs test is NEGATIVE. Positive Coombs = immune hemolysis (AIHA). This distinguishes TTP from autoimmune hemolytic anemia.Q11. Which of the following lab findings is most consistent with TTP? A) Low fibrinogen, elevated D-dimer, prolonged PT B) Schistocytes, elevated LDH, normal PT/aPTT, negative Coombs C) Positive Coombs, spherocytes, elevated indirect bilirubin D) Thrombocytosis, elevated PT, normal LDH ✅ B) Schistocytes, elevated LDH, normal PT/aPTT, negative Coombs | This combination = MAHA (schistocytes + LDH + bilirubin + haptoglobin) with intact coagulation and non-immune hemolysis — classic TTP profile. LDH often >1000 IU/dL in TTP.Q12. The mainstay of treatment for acquired TTP is: A) Platelet transfusion B) Fresh frozen plasma infusion alone C) Therapeutic plasma exchange (TPE) D) IV immunoglobulin ✅ C) Therapeutic plasma exchange (TPE) | TPE = MAINSTAY. Replacement fluid = FFP/thawed plasma (provides functional ADAMTS13 + removes autoantibodies). Before TPE: mortality ~90%. With TPE: remission >80%. Continue until platelet count normalises AND hemolysis resolved ≥2 days.Q13. In TTP, platelet transfusion is: A) First-line treatment B) Relatively contraindicated C) Recommended if platelets <50,000/µL D) Used to prevent bleeding routinely ✅ B) Relatively contraindicated | Platelet transfusion is CONTRAINDICATED in TTP — providing more platelets "fuels the fire" by worsening microvascular occlusions. Only given if severe life-threatening bleeding or invasive procedure needed (platelets <10,000/µL).Q14. Caplacizumab, used in TTP, has what mechanism of action? A) Anti-CD20 antibody that depletes B cells B) Anti-vWF nanobody that blocks vWF-GPIb-IX-V interaction C) Inhibitor of complement factor H D) Recombinant ADAMTS13 enzyme ✅ B) Anti-vWF nanobody that blocks vWF-GPIb-IX-V interaction | Caplacizumab = humanized single variable domain immunoglobulin (nanobody) against vWF → blocks vWF binding to platelet GPIb-IX-V receptor → prevents platelet aggregation. HERCULES trial: faster platelet recovery, fewer TPE days, ↓30-day relapse.Q15. Which drug is most strongly associated with antibody-mediated TTP with severe ADAMTS13 deficiency? A) Aspirin B) Gemcitabine C) Ticlopidine D) Cyclosporine ✅ C) Ticlopidine | Ticlopidine causes Ab-mediated TTP with ADAMTS13 deficiency in 80-90% of cases. Clopidogrel — few cases, Ab-mediated. Gemcitabine, Cyclosporine, Mitomycin C, Tacrolimus = endothelial toxicity type — ADAMTS13 is NORMAL — discontinue drug.Q16. Organs most commonly affected by microvascular thrombosis in TTP include all EXCEPT: A) Brain B) Kidney C) Heart D) Large bowel (colon) ✅ D) Large bowel (colon) | TTP microthrombi predominantly affect: Brain (neurologic symptoms), Kidney, Heart, Adrenals, Pancreas. The colon is not a primary target organ. In contrast, typical HUS (Shiga toxin) affects the colon and kidneys predominantly.Q17. A patient with TTP on PLASMIC score assessment scores 7 points. What is the next best step? A) Observe and repeat labs in 24 hours B) Start anticoagulation C) Immediate therapeutic plasma exchange D) Give platelet transfusion ✅ C) Immediate therapeutic plasma exchange | PLASMIC score 6-7 = HIGH risk for acquired TTP → immediate plasma exchange WITHOUT waiting for ADAMTS13 results. Score 5 = intermediate (consult hematology, consider PEX). Score 0-4 = low risk, consider alternative diagnosis.Q18. In TTP, which vWF receptor on platelets mediates pathological platelet aggregation? A) GPIIb-IIIa B) GPIb-IX-V C) GPVI D) P-selectin ✅ B) GPIb-IX-V | UL-vWF multimers (accumulated due to ADAMTS13 deficiency) bind platelet GPIb-IX-V receptor under shear stress → pathological platelet aggregation → microthrombi. GPIIb-IIIa (fibrinogen receptor) is targeted by drugs like abciximab but is NOT the primary receptor in TTP.Q19. Which of the following statements about TTP morphology is CORRECT? A) Thrombi contain abundant fibrin and are seen in large vessels B) Vessel wall shows prominent inflammatory infiltrate C) Hyaline thrombi in arterioles and capillaries with no vessel wall inflammation D) Microthrombi are restricted to the renal glomeruli only ✅ C) Hyaline thrombi in arterioles and capillaries with no vessel wall inflammation | TTP morphology: hyaline (platelet + vWF rich) microthrombi in arterioles and capillaries of multiple organs | NO vessel wall inflammation (distinguishes from vasculitis) | Onion-skin intimal thickening seen in some arterioles | Organs: brain, kidney, heart, adrenals, pancreas.Q20. The relapse rate of TTP after initial remission with plasma exchange is approximately: A) <5% B) 5-10% C) 25-45% within 30 days D) >70% within 1 week ✅ C) 25-45% within 30 days | TTP has a significant relapse rate: 25-45% relapse within 30 days of initial remission; 12-40% have late relapses. Risk is higher if ADAMTS13 activity remains persistently low (<10%). Rituximab (anti-CD20) reduces relapse risk when added to initial therapy.To import all 20 cards: Copy all 20 lines above → paste into a single
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