Hematopathology — MCQs

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 1181: Helmet cells are characteristic of which of the following conditions?

A. Hemolytic uremic syndrome (Correct Answer)
B. Polysplenia
C. Spherocytosis
D. Acanthocytosis

Explanation: **Explanation:** **Helmet cells**, also known as **schistocytes**, are fragmented red blood cells that result from mechanical damage as they pass through obstructed or narrowed small blood vessels [1][3]. 1. **Why Option A is Correct:** **Hemolytic Uremic Syndrome (HUS)** is a type of **Microangiopathic Hemolytic Anemia (MAHA)**. In HUS, endothelial injury leads to the formation of microthrombi (platelet plugs) in small vessels [2]. As RBCs attempt to squeeze through these fibrin-rich meshes, they are "sliced" or fragmented, resulting in helmet-shaped cells (schistocytes) [1][3]. This is a hallmark finding on a peripheral blood smear. 2. **Why Other Options are Incorrect:** * **B. Polysplenia:** This is a congenital syndrome associated with multiple small spleens. It typically presents with **Howell-Jolly bodies** (nuclear remnants) due to splenic dysfunction, not fragmented cells. * **C. Spherocytosis:** Characterized by **Spherocytes** (small, dark RBCs lacking central pallor). This is due to a molecular defect in membrane proteins (like Spectrin or Ankyrin), not mechanical fragmentation. * **D. Acanthocytosis:** Characterized by **Acanthocytes** (spur cells with irregular projections). These are seen in Abetalipoproteinemia or severe liver disease due to altered membrane lipids. **High-Yield Clinical Pearls for NEET-PG:** * **MAHA Differential:** Always look for schistocytes/helmet cells in **HUS, TTP (Thrombotic Thrombocytopenic Purpura), and DIC (Disseminated Intravascular Coagulation) [3].** * **HUS Triad:** Microangiopathic hemolytic anemia, Thrombocytopenia, and Acute Renal Failure [1]. * **Other causes of Schistocytes:** Malignant hypertension and prosthetic heart valves (mechanical hemolysis). **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 540-541. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 946-948. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 667-668.

Question 1182: Which of the following acute-phase reactants is responsible for the anemia associated with chronic inflammation?

A. Fibrinogen
B. Serum amyloid A (SAA)
C. C-reactive protein (CRP)
D. Hepcidin (Correct Answer)

Explanation: **Explanation:** The correct answer is **Hepcidin**. **Mechanism of Anemia of Chronic Disease (ACD):** In the setting of chronic inflammation, pro-inflammatory cytokines (primarily **IL-6**) stimulate the liver to synthesize **Hepcidin**, a key iron-regulatory hormone [1]. Hepcidin induces the degradation of **ferroportin**, the only known iron exporter on the surface of enterocytes and macrophages [1]. This leads to: 1. **Decreased intestinal iron absorption.** 2. **Sequestration of iron** within the reticuloendothelial system (macrophages) [1]. Consequently, iron is "trapped" and unavailable for erythropoiesis, leading to a functional iron deficiency despite adequate total body iron stores. **Analysis of Incorrect Options:** * **A. Fibrinogen:** An acute-phase reactant that promotes erythrocyte sedimentation (ESR) by causing "rouleaux" formation, but it does not affect iron metabolism. * **B. Serum amyloid A (SAA):** An apolipoprotein associated with chronic inflammation; its prolonged elevation can lead to secondary (AA) amyloidosis, not anemia. * **C. C-reactive protein (CRP):** A sensitive but non-specific marker of systemic inflammation used to monitor disease activity; it has no direct role in the pathogenesis of anemia. **High-Yield Clinical Pearls for NEET-PG:** * **Hallmark Lab Findings in ACD:** Low Serum Iron, **High Ferritin** (as it is also an acute-phase reactant), and **Low TIBC** (Total Iron Binding Capacity). * **Hepcidin vs. Ferroportin:** Remember, Hepcidin is the "brake" on iron flow; high Hepcidin = Low serum iron [1]. * **Treatment:** The primary treatment for ACD is managing the underlying inflammatory condition. Erythropoietin (EPO) levels are often inappropriately low for the degree of anemia in these patients [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. 658-662.

Question 1183: Alpha-naphthyl esterase enzyme is present in which type of leukocyte?

A. Neutrophil
B. Eosinophil
C. Lymphocyte
D. Monocyte (Correct Answer)

Explanation: **Explanation:** The correct answer is **Monocyte**. **1. Why Monocyte is correct:** Alpha-naphthyl esterase (ANE) is a **non-specific esterase (NSE)**. These enzymes are characteristic markers for cells of the **monocytic lineage**. In cytochemistry, NSE staining is used to differentiate Acute Myeloid Leukemia (AML) subtypes. Monoblasts and monocytes show strong, diffuse positivity for ANE, which is characteristically **inhibited by the addition of sodium fluoride (NaF)**. This is a crucial diagnostic feature for AML-M4 (Myelomonocytic) and AML-M5 (Monocytic) in the FAB classification. **2. Why other options are incorrect:** * **Neutrophils:** These cells are primarily identified by **Myeloperoxidase (MPO)** and **Specific Esterase** (Naphthol AS-D Chloroacetate esterase). They are generally negative for non-specific esterases like ANE. * **Eosinophils:** These are identified by their distinct granules and are MPO positive. They do not typically express diagnostic levels of ANE. * **Lymphocytes:** While some T-cells may show focal, "dot-like" positivity for ANE, it is not their characteristic marker. Diffuse cytoplasmic staining is specific to monocytes. **3. High-Yield Clinical Pearls for NEET-PG:** * **MPO:** Best marker for **Myeloid** differentiation (AML). * **Specific Esterase (CAE):** Marks **Granulocytic** series (Neutrophils). * **Non-Specific Esterase (ANE/ANAE):** Marks **Monocytic** series; inhibited by **Sodium Fluoride**. * **PAS (Periodic Acid Schiff):** Shows "block positivity" in **L1/L2 ALL** and "smudgy/diffuse" positivity in **AML-M6** (Erythroleukemia). * **LAP Score (Leukocyte Alkaline Phosphatase):** Elevated in Leukemoid reaction; decreased in **CML**.

Question 1184: Warm-antibody immunohemolytic anemia is seen in all of the following conditions except?

A. Systemic lupus erythematosus
B. Alpha-methyl-dopa ingestion
C. Quinidine
D. Infectious mononucleosis (Correct Answer)

Explanation: Explanation: Immunohemolytic anemias are classified based on the thermal reactivity of the antibodies involved. **Warm-antibody AIHA** is mediated by **IgG** antibodies that react optimally at 37°C [1], whereas **Cold-antibody AIHA** is mediated by **IgM** antibodies reacting below 30°C. **Why Infectious Mononucleosis is the correct answer:** Infectious mononucleosis (caused by EBV) is a classic cause of **Cold Agglutinin Disease**. It triggers the production of IgM antibodies, specifically with **anti-i specificity**. These antibodies bind to RBCs in the cooler peripheral circulation, leading to complement fixation and extravascular hemolysis. Therefore, it is not associated with warm antibodies. **Analysis of incorrect options (Causes of Warm AIHA):** * **Systemic Lupus Erythematosus (SLE):** This is the most common autoimmune cause of warm AIHA. It involves IgG-mediated destruction of RBCs, often leading to spherocyte formation [1]. * **Alpha-methyl-dopa:** This drug induces a true autoimmune state where the drug acts as a trigger for the body to produce IgG antibodies against intrinsic RBC antigens (specifically the Rh system) [2]. * **Quinidine:** While often associated with "innocent bystander" (Type II hypersensitivity) mechanisms, it typically presents as an acute hemolytic process involving IgG or IgM that reacts at warm temperatures, traditionally categorized under the warm/drug-induced spectrum in standard pathology texts [2]. **NEET-PG High-Yield Pearls:** * **Warm AIHA:** IgG, Extravascular hemolysis (Splenic macrophages), Spherocytes on smear. Associated with CLL, SLE, and drugs (α-methyldopa, Penicillin) [1]. * **Cold AIHA:** IgM, Intravascular/Extravascular hemolysis, RBC Clumping/Agglutination on smear. Associated with *Mycoplasma pneumoniae* (anti-I) and Infectious Mononucleosis (anti-i). * **Direct Coombs Test:** The gold standard for diagnosing both types [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 602-603. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 651-652.

Question 1185: Disseminated intravascular coagulation (DIC) is commonly associated with which subtype of Acute Myeloid Leukemia (AML)?

A. Monocytic (M5)
B. Promyelocytic (M3) (Correct Answer)
C. Erythrocytic (M6)
D. Megakaryocytic (M7)

Explanation: **Explanation:** **Correct Answer: B. Promyelocytic (M3)** Acute Promyelocytic Leukemia (APL), classified as FAB M3, is characterized by the translocation **t(15;17)**, involving the *PML-RARA* fusion gene [1]. The primary reason for its strong association with **Disseminated Intravascular Coagulation (DIC)** is the presence of numerous primary granules in the malignant promyelocytes [1]. These granules contain **procoagulants and tissue factor-like substances** (thromboplastin) that, when released, trigger the extrinsic coagulation cascade [2]. Additionally, these cells express annexin II, which accelerates fibrinolysis. This "double hit" of coagulation activation and hyperfibrinolysis makes DIC a life-threatening emergency in M3 patients, often exacerbated by the initiation of chemotherapy. **Incorrect Options:** * **A. Monocytic (M5):** More commonly associated with **extramedullary involvement**, such as gingival hyperplasia, skin infiltration (leukemia cutis), and CNS involvement [1]. * **C. Erythrocytic (M6):** Characterized by the proliferation of erythroid precursors; it does not typically present with coagulopathy. * **D. Megakaryocytic (M7):** Frequently associated with **acute myelofibrosis** and is common in children with Down Syndrome (under age 5). **High-Yield Clinical Pearls for NEET-PG:** * **Auer Rods:** M3 shows "faggot cells" (bundles of Auer rods) [1]. * **Treatment:** All-trans retinoic acid (ATRA) or Arsenic Trioxide (ATO) are used to induce differentiation of promyelocytes, which helps resolve the DIC. * **Morphology:** M3 cells are typically large with heavily granulated cytoplasm and "bilobed" or "butterfly" nuclei [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. 620-621. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 671-672.

Question 1186: Which stain is used for the diagnosis of granulocytic sarcoma?

A. Myeloperoxidase (Correct Answer)
B. Leucocyte alkaline phosphatase
C. Non specific esterase
D. Neuron specific esterase

Explanation: **Explanation:** **Granulocytic Sarcoma** (also known as **Chloroma** or Myeloid Sarcoma) is an extramedullary tumor mass composed of immature myeloid cells. It is most commonly associated with Acute Myeloid Leukemia (AML), particularly the M2, M4, and M5 subtypes. **Why Myeloperoxidase (MPO) is the correct answer:** MPO is the most sensitive and specific marker for cells of the **myeloid lineage**. Since granulocytic sarcoma consists of myeloblasts and maturing granulocytes, MPO staining (either via cytochemistry or immunohistochemistry) is the gold standard for confirming the myeloid nature of the tumor cells and differentiating it from lymphomas. **Analysis of Incorrect Options:** * **Leucocyte Alkaline Phosphatase (LAP):** This enzyme is found in mature neutrophils. The LAP score is used to differentiate Leukemoid Reaction (High score) from Chronic Myeloid Leukemia (Low score). It is not used to identify immature blasts in a sarcoma. * **Non-specific Esterase (NSE):** This stain is a marker for the **monocytic lineage**. While it may be positive in the myelomonocytic variant of myeloid sarcoma, MPO remains the primary diagnostic stain for the "granulocytic" type. * **Neuron Specific Enolase (NSE):** This is a marker for neuroendocrine tumors and small cell carcinomas, having no role in the diagnosis of myeloid neoplasms. **High-Yield Clinical Pearls for NEET-PG:** * **Common Site:** Often involves the bone, periosteum, soft tissues, and lymph nodes. * **Cytogenetics:** Frequently associated with **t(8;21)** [1]. * **Nomenclature:** The term "Chloroma" is derived from the greenish color of the tumor caused by the presence of the MPO enzyme itself. * **Other Markers:** CD33, CD117 (c-kit), and Lysozyme are also typically positive. **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. 620.

Question 1187: Basophilic stippling of red blood cells is a finding associated with which of the following enzymatic deficiencies?

A. ALA synthetase deficiency
B. Ferrochelatase deficiency
C. 5' nucleotidase deficiency (Correct Answer)
D. ALA dehydrogenase deficiency

Explanation: **Explanation:** **Basophilic stippling** refers to the presence of numerous fine or coarse blue granules within the cytoplasm of red blood cells on a peripheral smear. These granules represent **precipitated ribosomes and clusters of ribosomal RNA (rRNA).** **Why Option C is Correct:** In normal reticulocyte maturation, the enzyme **Pyrimidine 5' nucleotidase** is responsible for the degradation of ribosomal RNA. A deficiency of this enzyme (either congenital or acquired via lead poisoning) prevents the breakdown of RNA. This results in the persistence and precipitation of ribosomal fragments, which manifest as coarse basophilic stippling. **Analysis of Incorrect Options:** * **Option A (ALA synthetase):** This is the rate-limiting enzyme of heme synthesis. Its deficiency is associated with **Sideroblastic anemia**, characterized by Pappenheimer bodies (iron granules) rather than ribosomal stippling. * **Option B (Ferrochelatase):** This enzyme incorporates iron into protoporphyrin. Inhibition of ferrochelatase (as seen in lead poisoning or Erythropoietic Protoporphyria) leads to increased zinc protoporphyrin levels, but the stippling itself is due to the concurrent inhibition of 5' nucleotidase. * **Option D (ALA dehydrogenase):** Also known as Porphobilinogen (PBG) synthase. While inhibited in lead poisoning [1], its deficiency primarily leads to an accumulation of ALA, not the ribosomal precipitation seen in stippling. **NEET-PG High-Yield Pearls:** * **Lead Poisoning:** The most common cause of "coarse" basophilic stippling [1]. Lead inhibits both Ferrochelatase and Pyrimidine 5' nucleotidase. * **Differential Diagnosis:** Basophilic stippling is also seen in Thalassemia [2], Megaloblastic anemia, and Arsenic poisoning. * **Mnemonic:** "STIPPLED" – **S**ideroblastic anemia, **T**halassemia [2], **I**ron deficiency (rare), **P**yrimidine 5' nucleotidase deficiency, **P**lumbism (Lead) [1], **L**iver disease, **E**rythroleukemia, **D**yserythropoiesis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Infectious Diseases, pp. 418-419. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 648.

Question 1188: All of the following are acquired causes of hypercoagulability, except?

A. Infection (Correct Answer)
B. Inflammatory Bowel disease
C. Myeloproliferative disorders
D. Prolonged surgery > 1 hour

Explanation: **Explanation:** Hypercoagulability (thrombophilia) can be broadly classified into **Primary (Genetic/Inherited)** and **Secondary (Acquired)** causes [1]. **Why Option A is the correct answer:** While severe systemic infections (like sepsis) can trigger Disseminated Intravascular Coagulation (DIC) [3], **Infection** itself is generally considered a *trigger* or a transient state rather than a classic "acquired hypercoagulable state" in standard hematopathology classifications (such as Virchow’s Triad). In the context of this specific question, the other three options are well-documented, classic clinical conditions that predispose a patient to venous or arterial thrombosis over a sustained period. **Analysis of Incorrect Options:** * **B. Inflammatory Bowel Disease (IBD):** Patients with Crohn’s or Ulcerative Colitis are in a chronic pro-inflammatory state. Cytokines (TNF-α, IL-6) increase procoagulant factors (Factor VIII, Fibrinogen) and decrease natural anticoagulants (Protein S), making it a recognized acquired cause. * **C. Myeloproliferative Disorders (MPDs):** Conditions like Polycythemia Vera or Essential Thrombocythemia lead to increased blood viscosity and qualitative platelet abnormalities, significantly increasing the risk of thrombosis (e.g., Budd-Chiari syndrome). * **D. Prolonged Surgery (>1 hour):** Surgery is a major acquired risk factor due to prolonged immobilization (venous stasis), tissue trauma (releasing tissue factor), and the postoperative systemic inflammatory response. **NEET-PG High-Yield Pearls:** * **Most common inherited cause:** Factor V Leiden mutation (Activated Protein C resistance) [1]. * **Most common acquired cause:** Smoking and Obesity (General); Oral Contraceptive Pills (Young females); Malignancy (Trousseau sign). * **Antiphospholipid Antibody Syndrome (APS):** The only autoimmune condition that is a major acquired cause of both arterial and venous thrombosis [2]. * **Virchow’s Triad:** Endothelial injury, Stasis, and Hypercoagulability. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 133-134. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 134-135. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 672-673.

Question 1189: Autoimmune hemolytic anemia is seen in which of the following conditions?

A. All of the above
B. Acute Myeloid Leukemia (AML)
C. Chronic Myeloid Leukemia (CML)
D. Chronic Lymphocytic Leukemia (CLL) (Correct Answer)

Explanation: **Explanation:** **Chronic Lymphocytic Leukemia (CLL)** is the correct answer because it is the most common leukemia associated with autoimmune phenomena [1]. In CLL, the neoplastic B-cells are dysfunctional; they not only fail to produce effective antibodies but also act as antigen-presenting cells that trigger T-cell imbalance. This leads to the production of polyclonal autoantibodies by residual non-neoplastic B-cells against self-antigens on red blood cells (RBCs). Approximately 5–10% of CLL patients develop **Warm-type Autoimmune Hemolytic Anemia (AIHA)**, characterized by a positive Direct Antiglobulin Test (Coombs test) [2]. **Why other options are incorrect:** * **Acute Myeloid Leukemia (AML):** This is a proliferation of immature myeloid blasts. While it causes anemia due to bone marrow infiltration (myelophthisis), it is not typically associated with autoimmune-mediated destruction of RBCs. * **Chronic Myeloid Leukemia (CML):** CML is a myeloproliferative neoplasm driven by the BCR-ABL fusion gene. Anemia in CML is usually normocytic and results from the displacement of erythroid precursors by the massive expansion of the myeloid series, not autoimmunity. **NEET-PG High-Yield Pearls:** * **Evans Syndrome:** The combination of AIHA and Immune Thrombocytopenic Purpura (ITP), which can be seen in CLL patients. * **Richter Transformation:** The progression of CLL into Diffuse Large B-Cell Lymphoma (DLBCL), often signaled by sudden clinical worsening. * **Smudge Cells:** A classic peripheral smear finding in CLL due to the fragility of the neoplastic lymphocytes [1]. * **Drug-Induced AIHA:** Fludarabine, a drug used to treat CLL, can sometimes paradoxically worsen or trigger AIHA. **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. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 602-603.

Question 1190: Which is considered the intermediate form of Non-Hodgkin's lymphoma?

A. Small noncleaved cell
B. Diffuse, small cleaved cell (Correct Answer)
C. Lymphoblastic
D. Large cell immunoblastic

Explanation: This question refers to the **Working Formulation for Clinical Usage**, a historical but high-yield classification system for Non-Hodgkin Lymphomas (NHL) that categorizes them based on clinical aggressiveness into Low, Intermediate, and High grades. ### **Explanation of the Correct Answer** **B. Diffuse, small cleaved cell:** Under the Working Formulation, lymphomas are categorized by their architectural pattern (follicular vs. diffuse) and cell type. While follicular lymphomas are generally low-grade, **diffuse** patterns often shift the prognosis [1]. Specifically, **Diffuse, small cleaved cell lymphoma** is classified as an **Intermediate-grade** lymphoma. It represents a more aggressive clinical course than its follicular counterpart but is less rapidly fatal than high-grade types. ### **Analysis of Incorrect Options** * **A. Small noncleaved cell:** This includes Burkitt’s and non-Burkitt’s types. These are characterized by extremely high mitotic rates and are classified as **High-grade** lymphomas. * **C. Lymphoblastic:** This is a highly aggressive neoplasm (often T-cell origin in the mediastinum) and is categorized as **High-grade** [2]. * **D. Large cell immunoblastic:** This is a subtype of diffuse large B-cell lymphoma (DLBCL) with prominent nucleoli and abundant cytoplasm. It is categorized as **High-grade** due to its rapid doubling time [3]. ### **NEET-PG High-Yield Pearls** * **Low Grade:** Follicular (small cleaved), Follicular (mixed), and Small Lymphocytic (SLL). * **Intermediate Grade:** Follicular (large cell), Diffuse (small cleaved), Diffuse (mixed), and Diffuse (large cell). * **High Grade:** Large cell immunoblastic, Lymphoblastic, and Small non-cleaved (Burkitt’s). * **Concept Check:** In the modern WHO classification, "Intermediate grade" is no longer a formal term, but for exam purposes, remember that **Diffuse** architecture generally upgrades the severity compared to **Follicular** architecture. **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. 610-612. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 560-561. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 563-564.

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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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