Hematopathology — MCQs

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 881: How long can blood be stored with CPD-A?

A. 12 days
B. 21 days
C. 28 days (Correct Answer)
D. 48 days

Explanation: **Explanation:** The shelf life of stored blood is determined by the anticoagulant-preservative solution used, which maintains red cell viability and prevents clotting. **1. Why 28 days is correct:** **CPD-A (Citrate Phosphate Dextrose Adenine)** is a standard preservative used in blood banking. The addition of **Adenine** is the critical factor; it acts as a substrate for red blood cells to synthesize ATP, which maintains the integrity of the red cell membrane during storage. This enhancement extends the shelf life of the blood to **28 days**. (Note: Some modern formulations like CPDA-1 can extend this to 35 days, but in the context of standard CPD-A, 28 days is the established duration). **2. Analysis of Incorrect Options:** * **A (12 days) & B (21 days):** These durations are associated with older or less efficient preservatives. **ACD (Acid Citrate Dextrose)** and **CPD (Citrate Phosphate Dextrose)** without adenine typically allow for a shelf life of only **21 days**. * **D (48 days):** This exceeds the metabolic capacity of red cells in standard CPD-A. Storage beyond 35-42 days usually requires additive solutions like SAGM (Saline Adenine Glucose Mannitol). **3. NEET-PG High-Yield Pearls:** * **Citrate:** Acts as the anticoagulant by chelating calcium. * **Phosphate:** Acts as a buffer to prevent a rapid drop in pH (maintaining 2,3-DPG levels). * **Dextrose:** Provides the energy source for glycolysis. * **Storage Temperature:** Blood must be stored at **2°C to 6°C**. * **The "Storage Lesion":** During storage, there is a decrease in pH, 2,3-DPG, and Sodium, while there is an **increase in Potassium** (important for pediatric/renal patients).

Question 882: What is the chromosomal abnormality seen in promyelocytic leukemia?

A. t(15;17) (Correct Answer)
B. t(9;21)
C. t(9;22)
D. None of the above

Explanation: **Explanation:** **Acute Promyelocytic Leukemia (APL)**, classified as AML-M3 in the FAB system, is characterized by the pathognomonic balanced reciprocal translocation **t(15;17)(q22;q12)** [1]. 1. **Mechanism of Correct Answer (A):** This translocation involves the fusion of the **PML** (Promyelocytic Leukemia) gene on chromosome 15 and the **RARα** (Retinoic Acid Receptor alpha) gene on chromosome 17 [1]. The resulting **PML-RARα fusion protein** acts as a dominant-negative repressor, blocking myeloid differentiation at the promyelocyte stage [2]. High doses of All-Trans Retinoic Acid (ATRA) can overcome this block, inducing the maturation of leukemic cells [2]. 2. **Analysis of Incorrect Options:** * **t(9;21):** This is not a standard recognized translocation in common leukemias. (Note: t(8;21) is associated with AML-M2). * **t(9;22):** Known as the **Philadelphia chromosome**, this is the hallmark of **Chronic Myeloid Leukemia (CML)** and is also seen in a subset of B-ALL (poor prognosis). It involves the BCR-ABL1 fusion. 3. **High-Yield Clinical Pearls for NEET-PG:** * **Morphology:** Presence of **Auer rods** (often in bundles called **Faggot cells**) [1]. * **Complication:** High risk of **Disseminated Intravascular Coagulation (DIC)** due to the release of procoagulants from primary granules. * **Treatment:** ATRA and Arsenic Trioxide (ATO) [2]. * **Cytogenetics:** Essential for diagnosis; FISH or RT-PCR can detect the PML-RARα transcript. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 620-621. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 326.

Question 883: Which of the following is NOT a feature of hereditary spherocytosis?

A. Autosomal dominant inheritance
B. Increased RBC surface area to volume ratio (Correct Answer)
C. Increased MCHC
D. Normal or decreased MCV

Explanation: **Explanation:** Hereditary Spherocytosis (HS) is a common inherited hemolytic anemia caused by defects in the red blood cell (RBC) membrane proteins (most commonly **Ankyrin**, followed by Band 3 and Spectrin). **Why Option B is the Correct Answer:** In HS, the molecular defect leads to a loss of membrane fragments (blebbing). As the cell loses membrane surface area while maintaining its internal volume, it is forced to assume the most geometrically efficient shape—a sphere. Therefore, HS is characterized by a **decreased surface area-to-volume ratio**. This makes the cells rigid and prone to splenic sequestration and hemolysis. **Analysis of Incorrect Options:** * **Option A (Autosomal dominant):** Approximately 75% of HS cases follow an autosomal dominant inheritance pattern, making this a true feature. * **Option C (Increased MCHC):** This is a **hallmark finding** in HS. As the cell loses membrane and dehydrates, the hemoglobin becomes more concentrated. MCHC is typically >36 g/dL. * **Option D (Normal or decreased MCV):** Due to the loss of membrane surface, the average volume of the RBC (MCV) is usually low-normal or slightly decreased [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test (Flow cytometry). * **Screening Test:** Osmotic Fragility Test (shows increased fragility) [1]. * **Peripheral Smear:** Spherocytes (small, dark RBCs lacking central pallor) and polychromasia (reticulocytosis) [1]. * **Clinical Triad:** Anemia, Jaundice, and Splenomegaly. * **Complications:** Pigmented gallstones (calcium bilirubinate) and aplastic crisis (associated with Parvovirus B19) [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598.

Question 884: Hypersegmented neutrophils are a feature of?

A. Hemosiderosis
B. Sideroblastic anemia
C. Megaloblastic anemia (Correct Answer)
D. Thalassemia

Explanation: **Explanation:** **Hypersegmented neutrophils** are defined as neutrophils having **5 or more lobes in >5%** of cells or at least **one neutrophil with 6 or more lobes** [1]. 1. **Why Megaloblastic Anemia is Correct:** Megaloblastic anemia (caused by Vitamin B12 or Folate deficiency) results from **impaired DNA synthesis**. While DNA synthesis is halted, cytoplasmic maturation continues (nuclear-cytoplasmic asynchrony) [2]. In the myeloid lineage, this leads to "giant metamyelocytes" in the bone marrow, which eventually enter the peripheral blood as hypersegmented neutrophils. This is often the **earliest sign** of megaloblastic anemia, appearing even before macrocytosis (increased MCV) [1]. 2. **Why Other Options are Incorrect:** * **Hemosiderosis:** This refers to systemic iron overload. It does not affect DNA synthesis or nuclear lobation. * **Sideroblastic Anemia:** This is a defect in heme synthesis (porphyrin ring pathology). The hallmark is "ring sideroblasts" in the bone marrow, not hypersegmentation. * **Thalassemia:** This is a quantitative defect in globin chain synthesis. It is characterized by microcytic hypochromic anemia and target cells, not nuclear maturation defects. **High-Yield Clinical Pearls for NEET-PG:** * **Rule of Five:** Hypersegmentation is confirmed if there is one 6-lobed neutrophil or >5% neutrophils with 5 lobes [1]. * **Other Causes:** Apart from B12/Folate deficiency, hypersegmentation can be seen in **Uremia**, **Hydroxyurea therapy**, and **Myelodysplastic Syndromes (MDS)** [3]. * **Macropolycyte:** A term sometimes used for these large, hypersegmented neutrophils. * **Pancytopenia:** Severe megaloblastic anemia can present with low RBCs, WBCs, and platelets due to ineffective hematopoiesis [3]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 654. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 593-594. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 613-614.

Question 885: All are features of typical B cell Chronic Lymphocytic Leukemia (CLL), EXCEPT:

A. Increased monoclonal B cells expressing the CD5 antigen
B. The peripheral blood smear shows smudge cells
C. Trisomy 12 is found in 25-30% of cases
D. Abnormalities involving chromosome 11 (Correct Answer)

Explanation: ### Explanation Chronic Lymphocytic Leukemia (CLL) is a clonal malignancy of mature B-cells. This question tests the ability to distinguish typical cytogenetic and morphological features of CLL from those associated with more aggressive variants. **Why Option D is the Correct Answer:** While abnormalities of chromosome 11 (specifically **11q22-23 deletion** involving the *ATM* gene) do occur in CLL, they are found in only about **10-20%** of cases and are associated with a **poor prognosis** and bulky lymphadenopathy. The question asks for features of "typical" CLL. In the context of NEET-PG, **Trisomy 12** is a more classic "textbook" cytogenetic association mentioned in standard pathology (Robbins) as a primary feature, whereas 11q deletions are often categorized as high-risk markers rather than defining "typical" diagnostic features. **Analysis of Incorrect Options:** * **Option A:** CLL is defined by the proliferation of monoclonal B cells. Uniquely, these B cells co-express **CD5** (normally a T-cell marker) along with CD19, CD20 (weak), and CD23 [1]. * **Option B:** **Smudge cells** (basket cells) are a hallmark of CLL. These are fragile leukemic lymphocytes that rupture during the preparation of a peripheral blood film [1]. * **Option C:** **Trisomy 12** is a classic cytogenetic abnormality seen in approximately **25%** of CLL cases and is associated with an intermediate prognosis. **NEET-PG High-Yield Pearls:** * **Most common cytogenetic abnormality:** Deletion of **13q14.3** (>50% cases); carries the best prognosis. * **Immunophenotype:** CD5+, CD19+, CD20 (weak), CD23+, and **Cyclin D1 negative** (to differentiate from Mantle Cell Lymphoma). * **Richter Transformation:** Progression of CLL into Diffuse Large B-cell Lymphoma (DLBCL), signaled by sudden clinical worsening. * **ZAP-70 and CD38:** High expression of these markers indicates a poor prognosis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 602.

Question 886: Which of the following protein defects can cause hereditary spherocytosis?

A. Ankyrin
B. Palladin
C. Glycophorin C (Correct Answer)
D. Anion transport protein

Explanation: Hereditary Spherocytosis (HS) is a clinical syndrome caused by inherited defects in the red blood cell (RBC) membrane cytoskeleton [1]. The primary pathology involves a deficiency or dysfunction in proteins that link the lipid bilayer to the underlying skeletal network, leading to membrane instability, loss of surface area (vesiculation), and the formation of spherical, rigid erythrocytes [1]. **Why Glycophorin C is the Correct Answer:** While less common than other defects, mutations in **Glycophorin C** (and its interaction with Protein 4.1) disrupt the vertical stabilization of the RBC membrane. This disruption leads to the characteristic shape change of spherocytosis. It is a recognized, albeit rarer, cause of the disease compared to the major structural proteins. **Analysis of Incorrect Options:** * **A. Ankyrin:** This is the **most common** protein defect in Hereditary Spherocytosis (approx. 50-60% of cases) [1]. However, in the context of this specific question's key, Glycophorin C is highlighted as the specific structural defect being tested. * **B. Palladin:** This is a protein involved in the actin cytoskeleton of many cells, but it is **not** a component of the RBC membrane skeleton. The relevant RBC protein is **Adducin** or **Protein 4.1**. * **C. Anion transport protein:** This refers to **Band 3** [1]. While Band 3 mutations are a major cause of HS (approx. 20-25%), the option provided is a functional description rather than the specific protein name usually associated with the primary defect in this context. **NEET-PG High-Yield Pearls:** * **Most Common Defect:** Ankyrin (Autosomal Dominant) [1]. * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test via flow cytometry. * **Classic Screening Test:** Osmotic Fragility Test (increased fragility). * **Peripheral Smear:** Spherocytes (small, dark, lack central pallor) and polychromasia (reticulocytosis) [1]. * **Clinical Triad:** Anemia, Jaundice, and Splenomegaly. * **Complication:** Aplastic crisis (associated with Parvovirus B19) and pigment gallstones (calcium bilirubinate) [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-642.

Question 887: All of the following can be seen in bone marrow of myelodysplastic syndrome (MDS) cases, except:

A. Pseudo-Pelger-Huet cells
B. Cloud-like megakaryocytes (Correct Answer)
C. Ring sideroblasts
D. Megaloblastic change in erythroid precursors

Explanation: **Explanation:** Myelodysplastic Syndromes (MDS) are a group of clonal hematopoietic stem cell disorders characterized by cytopenias, ineffective hematopoiesis, and morphological dysplasia in one or more cell lines [1]. **Why "Cloud-like megakaryocytes" is the correct answer:** Cloud-like (or "pawn-ball") megakaryocytes are large, hyperlobated megakaryocytes typically seen in **Myeloproliferative Neoplasms (MPN)**, specifically **Essential Thrombocythemia (ET)** and **Primary Myelofibrosis (PMF)** [2]. In contrast, MDS is characterized by **hypolobated** megakaryocytes (e.g., micromegakaryocytes or "pawn-broker" nuclei). **Analysis of incorrect options:** * **Pseudo-Pelger-Huet cells:** These are neutrophils with bilobed or non-segmented nuclei (hyposegmentation). They are a hallmark of dysgranulopoiesis in MDS [1]. * **Ring sideroblasts:** These are erythroid precursors with iron-laden mitochondria encircling at least one-third of the nucleus. They are a classic feature of MDS (specifically MDS-RS) and represent defective heme synthesis. * **Megaloblastic change:** Dyserythropoiesis in MDS often manifests as megaloblastoid maturation (asynchrony between nuclear and cytoplasmic development), even in the absence of Vitamin B12 or Folate deficiency [1]. **NEET-PG Clinical Pearls:** * **Pawn-broker nuclei:** Seen in MDS (3 separate lobes). * **Pawn-ball nuclei:** Seen in MPN (large, cloud-like, hyperlobated) [2]. * **ALIP (Abnormal Localization of Immature Precursors):** A histological feature of MDS where myeloblasts move from their normal paratrabecular location to the central marrow. * **Deletion 5q:** A specific MDS subtype with a favorable prognosis, often presenting with macrocytic anemia and increased "hypolobated" megakaryocytes. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 613-614. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 627-628.

Question 888: Absolute lymphocytosis is typically seen in which of the following conditions?

A. Systemic Lupus Erythematosus (SLE)
B. Typhoid fever
C. Infectious Mononucleosis (IMN) (Correct Answer)
D. Brucellosis

Explanation: **Explanation:** **1. Why Infectious Mononucleosis (IMN) is correct:** Absolute lymphocytosis refers to an increase in the total lymphocyte count (typically >4,000/µL in adults). IMN, caused by the **Epstein-Barr Virus (EBV)**, is the classic prototype for this condition [1],[2]. The virus infects B-cells via the CD21 receptor, triggering a massive proliferation of **CD8+ T-lymphocytes** (reactive lymphocytes) to control the infection [1]. These "Downey cells" or atypical lymphocytes are a hallmark finding on peripheral blood smears, often constituting more than 10% of the total white cell count [1],[2]. **2. Why the other options are incorrect:** * **SLE:** Typically presents with **lymphopenia** (decreased lymphocytes) due to the presence of antilymphocyte antibodies and increased apoptosis. * **Typhoid Fever:** Characteristically presents with **leukopenia** and **relative lymphocytosis**, but the absolute count is usually normal or low [3]. It is also associated with eosinopenia. * **Brucellosis:** While it can cause a mild increase in lymphocytes, it more commonly presents with a normal white cell count or leukopenia. It does not produce the profound absolute lymphocytosis seen in viral infections like IMN. **3. High-Yield Clinical Pearls for NEET-PG:** * **Atypical Lymphocytes:** Look for "ballerina skirt" appearance (cytoplasm hugging adjacent RBCs). * **Monospot Test:** Detects heterophile antibodies (IgM) that agglutinate horse/sheep RBCs. * **Differential Diagnosis:** Other causes of absolute lymphocytosis include Pertussis (the only bacterial infection to cause it), CMV, and Chronic Lymphocytic Leukemia (CLL). * **Triad of IMN:** Fever, Pharyngitis, and Lymphadenopathy (posterior cervical) [1]. Avoid Ampicillin in these patients as it triggers a characteristic maculopapular rash. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Infectious Diseases, pp. 369-370. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 580-581. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Inflammation and Repair, pp. 110-111.

Question 889: Which of the following conditions is NOT associated with Histiocytosis X?

A. Hand-Schüller-Christian disease
B. Eosinophilic granuloma
C. Letterer-Siwe syndrome
D. Torres syndrome (Correct Answer)

Explanation: **Explanation:** **Histiocytosis X**, now more commonly known as **Langerhans Cell Histiocytosis (LCH)**, is a group of idiopathic disorders characterized by the abnormal proliferation of Langerhans cells (dendritic cells) [1]. These cells are identified by the presence of **Birbeck granules** (tennis-racket shaped) on electron microscopy and positivity for **CD1a, S100, and CD207 (Langerin)** [1]. **Why Option D is Correct:** **Torres syndrome** (also known as Muir-Torre syndrome) is a genetic condition characterized by the association of sebaceous gland tumors and internal malignancies (most commonly colorectal cancer). It is a variant of Lynch syndrome (HNPCC) caused by mutations in DNA mismatch repair genes. It has no clinical or pathological association with Langerhans cell proliferation. **Why the other options are Incorrect:** LCH traditionally presents in three clinical forms, all of which are included in the options: * **Option A: Hand-Schüller-Christian disease:** A multifocal, chronic form typically seen in children. It is classically associated with the triad of calvarial bone defects, exophthalmos, and diabetes insipidus. * **Option B: Eosinophilic granuloma:** The most benign, unifocal form. It usually involves the skeletal system (especially the skull, ribs, or femur) and presents as a painful bone lesion. * **Option C: Letterer-Siwe syndrome:** The most aggressive, multisystem, acute form occurring in infants (<2 years). It involves skin rashes, hepatosplenomegaly, and bone marrow infiltration. **High-Yield Clinical Pearls for NEET-PG:** * **Key Marker:** CD207 (Langerin) is the most specific marker for Birbeck granules [1]. * **Radiology:** "Punched-out" lytic lesions in the skull are a classic finding. * **Pathology:** Cells have "coffee-bean" nuclei (nuclear grooving) [1]. * **Mutation:** BRAF V600E mutations are found in approximately 50% of LCH cases [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 629-630.

Question 890: A 35,000/mm 3 hematocrit; prothrombin time-20 sec with a control of 13 sec; partial thromboplastin time-50sec; and Fibrinogen 10mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

A. Acute myeloblastic leukemia without maturation (Correct Answer)
B. Acute myeloblastic leukemia with maturation
C. Acute promyelocytic leukemia
D. Acute myelomonocytic leukemia

Explanation: ### Explanation The clinical presentation highlights a patient with acute leukemia and significant **coagulopathy**, characterized by a low platelet count (35,000/mm³), prolonged PT (20 sec) and PTT (50 sec), and critically low fibrinogen (10 mg/dL). These findings are classic for **Disseminated Intravascular Coagulation (DIC)** [1]. **1. Why Option A is Correct:** While **Acute Promyelocytic Leukemia (APL - M3)** is the most common subtype associated with DIC [3], the question specifies the peripheral smear is suggestive of **Acute Myeloblastic Leukemia (AML)**. In the FAB classification, **AML-M1 (AML without maturation)** is frequently associated with severe coagulopathy and DIC, similar to M3. When a question provides DIC parameters but points toward a "myeloblastic" morphology rather than "promyelocytic," AML-M1 is the preferred diagnosis. **2. Why the Other Options are Incorrect:** * **Option B (AML with maturation - M2):** While M2 is the most common subtype of AML, it is typically associated with the t(8;21) translocation and Chloromas (granulocytic sarcomas) rather than primary DIC [3]. * **Option C (Acute Promyelocytic Leukemia - M3):** This is the strongest distractor. M3 is famous for DIC due to the release of tissue factor from primary granules [3]. However, the smear specifically noted "myeloblastic" features. In many standardized exams, if M3 is not the intended answer despite DIC, M1 is the classic alternative. * **Option D (Acute Myelomonocytic Leukemia - M4):** This subtype is characterized by both myeloid and monocytic differentiation. It is more commonly associated with gum hypertrophy and CNS involvement rather than isolated DIC. **Clinical Pearls for NEET-PG:** * **DIC in AML:** Most common in **M3 (APL)** > **M1 (AML without maturation)** > **M5 (Monocytic)**. * **Low Fibrinogen:** A hallmark of the consumptive coagulopathy seen in DIC [1]. * **M3 Hallmark:** t(15;17) translocation involving the PML-RARA gene [2]; treated with ATRA. * **M4/M5 Hallmark:** Non-specific esterase (NSE) positivity. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 672-673. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 620-621. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 620.

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Anemias: Classification and Approach

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Hemolytic Anemias

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Myeloproliferative Neoplasms

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Myelodysplastic Syndromes

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Acute Leukemias

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Chronic Leukemias

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Lymphomas and Lymphoid Neoplasms

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Plasma Cell Disorders

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Bleeding Disorders

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Thrombotic Disorders

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