Hematopathology — MCQs

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 841: Which of the following is an example of a chronic myeloproliferative disorder?

A. Neutrophilic leukaemoid reaction
B. Essential thrombocythaemia (Correct Answer)
C. Plasmacytosis
D. Hairy cell leukaemia

Explanation: **Explanation:** **Chronic Myeloproliferative Neoplasms (MPNs)** are a group of clonal hematopoietic stem cell disorders characterized by the autonomous overproduction of one or more mature myeloid lineages (erythroid, granulocytic, or megakaryocytic) [1]. **1. Why Essential Thrombocythaemia (ET) is correct:** ET is a classic BCR-ABL1 negative MPN [2]. It is characterized by the primary proliferation of megakaryocytes in the bone marrow, leading to a sustained increase in circulating platelets (usually >450,000/µL) [3]. It is frequently associated with mutations in **JAK2 (V617F)**, **CALR**, or **MPL** genes [2]. **2. Why other options are incorrect:** * **Neutrophilic Leukemoid Reaction:** This is a **reactive** (non-neoplastic) increase in the white blood cell count (usually >50,000/µL) in response to infection, inflammation, or stress. It is not a clonal malignancy. * **Plasmacytosis:** This refers to an increased number of plasma cells in the marrow or blood. While it can be seen in malignancies like Multiple Myeloma, it is a **lymphoid** lineage disorder, not a myeloid one. * **Hairy Cell Leukaemia:** This is a mature **B-cell lymphoproliferative disorder** characterized by "hairy" cytoplasmic projections and TRAP positivity. It is not part of the MPN group. **High-Yield Clinical Pearls for NEET-PG:** * **WHO Classification of MPNs:** Includes Chronic Myeloid Leukaemia (CML), Polycythemia Vera (PV), Essential Thrombocythaemia (ET), and Primary Myelofibrosis (PMF) [2]. * **JAK2 V617F Mutation:** Present in ~95% of PV cases and ~50-60% of ET and PMF cases [5]. * **Complications:** MPNs carry a high risk of thrombosis, hemorrhage, and potential transformation into Acute Myeloid Leukaemia (AML) or myelofibrosis [4]. * **Distinguishing ET from Reactive Thrombocytosis:** Look for giant platelets and megakaryocyte clusters on bone marrow biopsy in ET [3]. **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. 624-625. [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, p. 624. [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, pp. 627-628. [4] 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. 626-627. [5] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615.

Question 842: Routine Rh typing includes testing for which antigen?

A. A antigen
B. B antigen
C. C antigen
D. D antigen (Correct Answer)

Explanation: **Explanation:** The Rh blood group system is the second most important system in transfusion medicine after ABO. It consists of approximately 50 different antigens, but the **D antigen** is the most significant [1]. **1. Why D antigen is the correct answer:** The D antigen is the most **immunogenic** of all non-ABO antigens [2]. Routine Rh typing specifically tests for the presence or absence of the D antigen on the surface of red blood cells [1]. * **Rh-positive:** Individuals who possess the D antigen [1]. * **Rh-negative:** Individuals who lack the D antigen [1]. Because of its high potential to cause alloimmunization (leading to Hemolytic Disease of the Fetus and Newborn or Hemolytic Transfusion Reactions), it is the only Rh antigen routinely screened for in blood banks [2]. **2. Why other options are incorrect:** * **Options A & B (A and B antigens):** These belong to the **ABO blood group system**, not the Rh system [1]. While they are tested during routine "blood grouping," they are distinct from "Rh typing." * **Option C (C antigen):** The C, c, E, and e antigens are other major antigens in the Rh system (Fisher-Race nomenclature) [1]. While clinically important in complex cross-matching or sensitized patients, they are **not** part of routine Rh typing because they are less immunogenic than the D antigen [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Weak D (Du) phenotype:** Some individuals have low D antigen expression. They are treated as **donors** if Rh-positive (to prevent sensitizing others) but as **recipients** if Rh-negative. * **Most common Rh genotype:** CDe/cde (R1r) [1]. * **Rh-null syndrome:** A rare condition where all Rh antigens are missing, leading to stomatocytosis and mild hemolytic anemia. * **Anti-D Immunoglobulin:** Given to Rh-negative mothers at 28 weeks and within 72 hours of delivery to prevent Rh isoimmunization [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 603-604, 627-628. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 469-470.

Question 843: Which is the diagnostic test for Disseminated Intravascular coagulation?

A. Prothrombin Time (PT)
B. D-Dimer assay (Correct Answer)
C. Activated Partial Thromboplastin Time (APTT)
D. Bleeding Time (BT)

Explanation: **Explanation:** **Disseminated Intravascular Coagulation (DIC)** is a complex thrombohemorrhagic disorder characterized by the systemic activation of the coagulation cascade, leading to widespread microvascular thrombi and the subsequent consumption of platelets and clotting factors. **Why D-Dimer is the Correct Answer:** The hallmark of DIC is the simultaneous activation of coagulation and **fibrinolysis**. As the body attempts to break down widespread fibrin clots, plasmin cleaves cross-linked fibrin. **D-Dimer** is a specific degradation product of cross-linked fibrin. It is considered the most sensitive and specific diagnostic marker for DIC because it indicates both thrombin generation and active fibrinolysis [1]. While no single test is gold-standard, an elevated D-Dimer in the clinical context of sepsis, trauma, or malignancy is highly suggestive of DIC [2]. **Analysis of Incorrect Options:** * **Prothrombin Time (PT) & APTT:** These are often prolonged in DIC due to the consumption of clotting factors [1]. However, they are non-specific and can be elevated in liver disease or Vitamin K deficiency. * **Bleeding Time (BT):** This measures platelet function and vascular integrity. While BT may be prolonged due to thrombocytopenia in DIC, it is an outdated, non-specific bedside test not used for definitive diagnosis. **High-Yield Clinical Pearls for NEET-PG:** * **Best Screening Test:** Platelet count (Thrombocytopenia is almost universal) [2]. * **Most Specific Test:** D-Dimer or Fibrin Degradation Products (FDPs) [1]. * **Peripheral Smear:** Look for **Schistocytes** (fragmented RBCs), indicating microangiopathic hemolytic anemia (MAHA) [2]. * **Fibrinogen Levels:** Low fibrinogen is specific but lacks sensitivity as it is an acute-phase reactant [2]. * **International Society on Thrombosis and Haemostasis (ISTH) Score:** Uses Platelets, D-Dimer, PT, and Fibrinogen to diagnose overt DIC. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 151-152. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 625-626.

Question 844: What is the most common site of histiocytosis?

A. Bone (Correct Answer)
B. Skin
C. Lung
D. Liver

Explanation: **Explanation:** **Langerhans Cell Histiocytosis (LCH)**, formerly known as Histiocytosis X, is a proliferative disorder of dendritic cells (Langerhans cells) [1]. **1. Why Bone is the Correct Answer:** Bone is the most frequently involved organ in LCH, occurring in approximately **80% of cases**. It typically presents as a **solitary osteolytic lesion** (Eosinophilic Granuloma), most commonly involving the **skull**, followed by the femur, ribs, and mandible. In pediatric populations, it is a classic cause of a "punched-out" radiolucent lesion on a skull X-ray. **2. Why Other Options are Incorrect:** * **B. Skin:** This is the second most common site (approx. 30-40%). It often presents as a seborrheic dermatitis-like rash, especially in the Letterer-Siwe disease variant. * **C. Lung:** While common in adult smokers (Pulmonary LCH), it is less frequent than bone involvement across the general disease spectrum. * **D. Liver:** Involvement of the liver, spleen, or bone marrow indicates "high-risk" multisystem disease (Letterer-Siwe) but is statistically less common than skeletal involvement. **3. NEET-PG High-Yield Pearls:** * **Pathognomonic Marker:** **Birbeck Granules** (tennis-racket shaped pentalaminar structures) seen on Electron Microscopy [1]. * **Immunohistochemistry (IHC):** Positive for **CD1a**, **S100**, and **Langerin (CD207)** [1]. Langerin is the most specific marker. * **Clinical Triad (Hand-Schüller-Christian disease):** Calvarial bone defects, Exophthalmos, and Diabetes Insipidus. * **Mutation:** Frequently associated with the **BRAF V600E** mutation (approx. 50% of 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 845: Differential diagnosis for pancytopenia with cellular bone marrow includes the following except?

A. Megaloblastic anemia
B. Myelodysplasia
C. Paroxysmal Nocturnal Hemoglobinuria
D. Congenital dyserythropoietic anemia (Correct Answer)

Explanation: **Explanation:** Pancytopenia with a cellular (hypercellular or normocellular) bone marrow indicates **ineffective hematopoiesis**, where the marrow is producing cells, but they are defective and undergo premature destruction before reaching the peripheral circulation. **Why Option D is the Correct Answer:** **Congenital Dyserythropoietic Anemia (CDA)** is a group of rare hereditary disorders characterized by ineffective erythropoiesis and macrocytosis. While the bone marrow is hypercellular, the defect is **lineage-specific**. CDA typically presents with **isolated anemia** and reticulocytopenia, not pancytopenia. While the marrow shows significant erythroid hyperplasia and morphological abnormalities (like binucleated precursors), the leukocyte and platelet lineages remain largely unaffected. **Analysis of Incorrect Options:** * **Megaloblastic Anemia:** The classic cause of pancytopenia with a hypercellular marrow. Vitamin B12/Folate deficiency leads to impaired DNA synthesis, causing "asynchrony" where cells die within the marrow (intramedullary hemolysis) [1]. * **Myelodysplastic Syndrome (MDS):** Characterized by "sick" stem cells. The marrow is usually hypercellular with dysplastic changes in all three cell lines, but peripheral cytopenias occur due to high rates of apoptosis [1]. * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** PNH is a stem cell disorder. While it can be associated with aplastic (hypocellular) marrow, it frequently presents with a cellular marrow during hemolytic phases or when evolving from/into MDS. **High-Yield Clinical Pearls for NEET-PG:** * **Pancytopenia + Hypocellular Marrow:** Aplastic Anemia, Hypoplastic MDS, Fanconi Anemia. * **Pancytopenia + Hypercellular Marrow:** Megaloblastic anemia (Most common) [1], MDS [1], Aleukemic Leukemia, PNH, Visceral Leishmaniasis (Kala-azar). * **CDA Hallmark:** Look for **"Internuclear bridges"** in CDA Type I and **"Gaucher-like cells"** or **"HEMPAS"** (Hereditary Erythroblastic Multinuclearity with Positive Acidified Serum test) in CDA Type II. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 592-614.

Question 846: Bence Jones proteins are:

A. Heavy chains
B. Heavy and light chains
C. Light chains (Correct Answer)
D. Immunoglobulins

Explanation: **Bence Jones proteins (BJP)** are monoclonal, free **immunoglobulin light chains** (either kappa or lambda) produced by neoplastic plasma cells [2]. In conditions like Multiple Myeloma, there is an overproduction of these light chains. Due to their low molecular weight (approx. 22-44 kDa), they are easily filtered by the renal glomerulus and excreted in the urine [1]. **Why the correct answer is right:** * **Option C:** BJP are specifically the **free light chains** of immunoglobulins [2]. Their unique biochemical property is their **heat solubility**: they precipitate when heated to 40–60°C and re-dissolve upon boiling (100°C), a classic diagnostic feature. **Why the incorrect options are wrong:** * **Option A & B:** Heavy chains are much larger molecules and are not typically excreted in the urine in this manner. While "Heavy Chain Disease" exists, it is a distinct clinical entity and not associated with Bence Jones proteinuria. * **Option D:** "Immunoglobulins" refers to the complete molecule (2 heavy + 2 light chains). Intact immunoglobulins are too large to be filtered by a healthy glomerulus; BJP represents only the light chain component. **High-Yield Clinical Pearls for NEET-PG:** * **Renal Impact:** BJP are nephrotoxic. They precipitate with Tamm-Horsfall protein in the distal tubules, forming "waxy/hard casts," leading to **Myeloma Kidney** (Cast Nephropathy) [1]. * **Detection:** BJP are **not detected by routine urine dipstick** (which primarily senses albumin). They are detected by the **Sulfosalicylic Acid (SSA) test** or confirmed via **Urine Protein Electrophoresis (UPEP)** showing an 'M' spike [3]. * **Amyloidosis:** Excess light chains can be processed by macrophages to form **AL Amyloid**, which stains with Congo Red and shows apple-green birefringence [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 942-943. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617. [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, pp. 608-609.

Question 847: In Acute Myeloid Leukemia (AML), which subtype is associated with the best prognosis?

A. Acute megakaryocytic leukemia
B. Acute monocytic leukemia
C. Acute promyelocytic leukemia (M3) (Correct Answer)
D. Erythroleukemia

Explanation: **Acute Promyelocytic Leukemia (APL/M3)** is considered the subtype with the best prognosis in AML due to its unique molecular target and highly effective targeted therapy [1]. It is characterized by the **t(15;17)** translocation, which fuses the *PML* gene with the *RARα* (Retinoic Acid Receptor alpha) gene [1]. This fusion protein blocks myeloid differentiation [1]. The introduction of **All-Trans Retinoic Acid (ATRA)** and **Arsenic Trioxide (ATO)** overcomes this block, inducing the differentiation of malignant promyelocytes into mature neutrophils. This targeted approach results in complete remission rates exceeding 90%, making it the most curable form of AML. **Analysis of Incorrect Options:** * **Acute Megakaryocytic Leukemia (M7):** Generally associated with a poor prognosis. It is frequently seen in children with Down Syndrome (under age 5), where the prognosis is slightly better, but in adults, it often presents with extensive marrow fibrosis and resistance to therapy. * **Acute Monocytic Leukemia (M5):** Associated with an intermediate to poor prognosis [1]. It has a high propensity for extramedullary involvement (e.g., gingival hyperplasia, CNS involvement) and a higher risk of relapse [1]. * **Erythroleukemia (M6):** Historically associated with a very poor prognosis, often arising from prior myelodysplastic syndromes (MDS) and carrying complex cytogenetic abnormalities that are resistant to standard chemotherapy. **High-Yield Clinical Pearls for NEET-PG:** * **DIC Risk:** M3 is a medical emergency due to the high risk of **Disseminated Intravascular Coagulation (DIC)** triggered by the release of procoagulants from primary granules (Auer rods) [1]. * **Morphology:** Look for **"Faggot cells"** (cells containing bundles of Auer rods) in M3 [1]. * **Differentiation Syndrome:** A potential complication of ATRA therapy characterized by fever, respiratory distress, and pulmonary infiltrates. **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.

Question 848: Which coagulation pathway(s) does the Prothrombin Time (PT) test evaluate?

A. Extrinsic and common pathway (Correct Answer)
B. Intrinsic and common pathway
C. Intrinsic pathway
D. Extrinsic pathway

Explanation: **Explanation:** The **Prothrombin Time (PT)** test measures the time it takes for plasma to clot after the addition of Tissue Factor (Factor III) and Calcium [1]. It is the primary screening test for the **Extrinsic and Common pathways** of the coagulation cascade [1]. 1. **Why Option A is correct:** The PT test specifically monitors the activity of **Factor VII** (Extrinsic pathway) and the shared factors of the **Common pathway: Factors X, V, II (Prothrombin), and I (Fibrinogen).** Because Factor VII has the shortest half-life of all clotting factors, PT is highly sensitive to early vitamin K deficiency and liver dysfunction. 2. **Why Options B and C are incorrect:** The **Intrinsic pathway** (Factors XII, XI, IX, VIII) is evaluated by the **Activated Partial Thromboplastin Time (aPTT)** test, not PT [1]. 3. **Why Option D is incorrect:** While PT does evaluate the extrinsic pathway, it cannot function without the common pathway. A deficiency in Factor X, V, or II will also result in a prolonged PT; therefore, the test encompasses both. **High-Yield Clinical Pearls for NEET-PG:** * **Warfarin Monitoring:** PT (reported as **INR**) is used to monitor Warfarin therapy because Warfarin inhibits Vitamin K-dependent factors (II, VII, IX, X), and Factor VII is the first to decline. * **Liver Disease:** PT is one of the best indicators of the liver's synthetic function. * **Mnemonic for PT vs. aPTT:** * **PeT** (3 letters) = Extrinsic pathway (shorter name). * **PiiTT** (4 letters) = Intrinsic pathway (longer name). * **Mixing Study:** If PT is prolonged, a mixing study is performed. If it corrects, it indicates a factor deficiency; if it doesn't, it indicates the presence of an inhibitor (e.g., Lupus anticoagulant). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 128-130.

Question 849: Which of the following is false regarding Chediak-Higashi syndrome?

A. It is inherited in an autosomal dominant pattern. (Correct Answer)
B. There is a defect in the fusion of phagosomes and lysosomes.
C. Abnormalities are present in melanocytes.
D. Platelet abnormalities are present.

Explanation: **Explanation:** **Chediak-Higashi Syndrome (CHS)** is a rare, multisystem disorder caused by a mutation in the **LYST gene** (Lysosomal Trafficking Regulator). 1. **Why Option A is the correct answer (False statement):** Chediak-Higashi syndrome is inherited in an **autosomal recessive** pattern, not autosomal dominant [1]. The mutation leads to defective vesicle fusion and intracellular protein trafficking, resulting in the formation of pathognomonic **giant granules** in various cells [1]. 2. **Why the other options are true:** * **Option B:** The primary cellular defect is the failure of **phagosome-lysosome fusion** [1]. This prevents the effective killing of phagocytosed bacteria, leading to recurrent pyogenic infections (primarily by *Staphylococcus* and *Streptococcus*). * **Option C:** Melanocytes are affected because **melanosomes** (specialized lysosomes) fail to distribute melanin properly [1]. This results in **oculocutaneous albinism**, a hallmark clinical feature. * **Option D:** Platelets contain "dense bodies" (delta granules) which are lysosome-like organelles. Defective trafficking leads to a **storage pool deficiency**, resulting in platelet dysfunction and bleeding tendencies [1]. **NEET-PG High-Yield Pearls:** * **Peripheral Smear:** Look for **giant peroxidase-positive granules** in neutrophils and precursors [1]. * **Clinical Tetrad:** Partial albinism, recurrent infections, peripheral neuropathy, and mild bleeding [1]. * **Accelerated Phase:** A life-threatening "lymphoma-like" syndrome characterized by hemophagocytic lymphohistiocytosis (HLH), hepatosplenomegaly, and pancytopenia. * **Diagnosis:** Confirmed by genetic testing (LYST gene) or seeing giant granules on a blood film [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 245-246.

Question 850: What is the expected reticulocyte count in hemolytic jaundice?

A. 0.50%
B. 1%
C. 1.50%
D. 2.50% (Correct Answer)

Explanation: **Explanation:** In **hemolytic jaundice**, the fundamental pathology is the premature destruction of red blood cells (RBCs). To compensate for this anemia, the bone marrow becomes hyperactive (erythroid hyperplasia) and releases immature RBCs, known as **reticulocytes**, into the peripheral blood [2]. **1. Why 2.50% is correct:** The normal adult reticulocyte count ranges from **0.5% to 1.5%**. In a state of active hemolysis, the bone marrow attempts to maintain homeostasis by increasing production. A reticulocyte count **above 2%** (reticulocytosis) is a hallmark of hemolytic anemia and acute blood loss [2]. Therefore, 2.50% is the only option that reflects this compensatory marrow response. **2. Why other options are incorrect:** * **0.50% (Option A) and 1% (Option B):** These values fall within the lower to mid-normal range. They would be expected in healthy individuals or in cases of jaundice not caused by hemolysis (e.g., obstructive jaundice). * **1.50% (Option C):** This is the upper limit of the normal range. While it is higher than 0.5%, it does not represent the significant "shift" or increase typically seen in a hemolytic crisis. **High-Yield Clinical Pearls for NEET-PG:** * **Reticulocyte Production Index (RPI):** In cases of anemia, the raw reticulocyte percentage can be misleading. The RPI (or Corrected Reticulocyte Count) is used to assess if the marrow response is truly adequate. An **RPI > 2-3** indicates a good marrow response (hemolysis/hemorrhage). * **Supravital Stains:** Reticulocytes are visualized using stains like **New Methylene Blue** or **Brilliant Cresyl Blue**, which highlight the ribosomal RNA (organelles) as a blue reticular network. * **Polychromasia:** On a standard Leishman or Romanowsky stain, reticulocytes appear as larger, bluish-grey RBCs (polychromatophilic cells) [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. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 640.

Practice by Chapter

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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