Hematopathology Practice Questions

Q: A 20-year-old female presents with a 5-day history of fatigue, low-grade fever, and sore throat. Physical examination reveals bilateral enlarged, tender cervical lymph nodes, an exudative tonsillitis, and an enlarged spleen. A complete blood cell count reveals the hemoglobin and platelet counts to be within normal limits. The total white blood cell count is increased to 9200 cells per uL. Examination of the peripheral blood reveals the presence of atypical mononuclear cells with abundant cytoplasm. These cells have peripheral condensation of the cytoplasm, which gives them a "ballerina ski" appearance. Which one of the listed findings is most likely to be present in this individual?

Heterophil antibodies in the serum. **Explanation:** The clinical presentation of fever, exudative tonsillitis, cervical lymphadenopathy, and splenomegaly in a young adult is classic for **Infectious Mononucleosis (IM)**, most commonly caused by the **Epstein-Barr Virus (EBV)** [1], [2]. The definitive clue in this question is the description of **"ballerina skirt" cells**. These are **atypical T-lymphocytes** (CD8+ T-cells) reacting against EBV-infected B-cells [1]. They are characterized by abundant, pale cytoplasm that "hugs" or indents around adjacent red blood cells, creating a scalloped border. The most common screening test for IM is the **Monospot test**, which detects **heterophil antibodies** (IgM antibodies that agglutinate sheep or horse RBCs). **Analysis of Incorrect Options:** * **Option A:** Birbeck granules (tennis-racket shaped) are pathognomonic for **Langerhans Cell Histiocytosis**, not viral infections. * **Option B:** Elevated urinary d-ALA is seen in **Lead poisoning** or **Acute Intermittent Porphyria**, which present with abdominal pain and neurological symptoms, not pharyngitis. * **Option C:** While Group A Streptococcus causes exudative tonsillitis, it does not typically cause splenomegaly or the presence of "ballerina skirt" atypical lymphocytes. **NEET-PG High-Yield Pearls:** * **Triad of IM:** Fever, Pharyngitis, and Lymphadenopathy (usually posterior cervical) [2]. * **Atypical Lymphocytes:** Also known as **Downey cells** [1]. * **Complication:** Avoid contact sports for 3–4 weeks due to the risk of **splenic rupture**. * **Drug Rash:** Administration of **Ampicillin or Amoxicillin** in a patient with IM often results in a characteristic maculopapular rash. * **Diagnosis:** Heterophil antibody test is specific but may be negative in the first week or in children <5 years.

Q: A 70-year-old male has been experiencing intermittent epistaxis, fatigue, and bone pain for the past 4 months. Laboratory investigations reveal serum calcium of 12 mg/dL. What is the most likely finding in a bone marrow aspirate from this patient?

Numerous plasma cells. **Explanation:** The clinical presentation of an elderly patient with **bone pain, fatigue, and hypercalcemia (12 mg/dL)** is highly suggestive of **Multiple Myeloma (MM)** [1]. In MM, the neoplastic proliferation of plasma cells in the bone marrow leads to the production of osteoclast-activating factors, causing lytic bone lesions (bone pain) and the release of calcium into the bloodstream (hypercalcemia). Fatigue is typically due to normocytic normochromic anemia [1]. * **Why Option B is correct:** The hallmark of Multiple Myeloma is the infiltration of the bone marrow by malignant **plasma cells** (usually >10%) [1]. These cells are characterized by an eccentric nucleus, a perinuclear halo (Golgi apparatus), and "clock-face" chromatin. * **Why Option A is incorrect:** Myeloid blasts are characteristic of **Acute Myeloid Leukemia (AML)**. While AML can cause fatigue and bone pain, hypercalcemia is much more common in MM. * **Why Option C is incorrect:** A 'dry tap' occurs when the marrow is fibrotic or hypercellular (e.g., Myelofibrosis or Hairy Cell Leukemia). While it can occur in MM, it is not the most "likely" diagnostic finding compared to plasma cell infiltration. * **Why Option D is incorrect:** Lymphoid blasts are seen in **Acute Lymphoblastic Leukemia (ALL)**, which primarily affects children, not 70-year-old males. **NEET-PG High-Yield Pearls:** * **CRAB Criteria for MM:** **C**alcium elevation, **R**enal insufficiency, **A**nemia, and **B**one lesions [1]. * **M-Spike:** Found on Serum Protein Electrophoresis (usually IgG or IgA) [1]. * **Bence-Jones Proteins:** Free light chains found in urine. * **Peripheral Smear:** Look for **Rouleaux formation** due to increased ESR [1]. * **Skull X-ray:** Classic "punched-out" lytic lesions [1].

Q: All of the following statements regarding primary effusion lymphoma are true EXCEPT?

The proliferating cells are NK cells. **Primary Effusion Lymphoma (PEL)** is a rare, aggressive B-cell non-Hodgkin lymphoma that primarily presents as malignant effusions in body cavities (pleural, pericardial, or peritoneal) without a detectable solid tumor mass [1]. ### Explanation of Options: * **Why Option C is the correct answer (False statement):** The proliferating cells in PEL are **B-cells**, not NK cells. Although these cells often lack traditional B-cell surface markers (CD19, CD20) due to their highly undifferentiated state, they demonstrate clonal immunoglobulin gene rearrangements, confirming their B-cell lineage. * **Option A (True):** PEL is also known as **body cavity lymphoma** because it characteristically grows as a liquid phase in the serous spaces. * **Option B (True):** **HHV-8 (Human Herpesvirus-8)**, also known as Kaposi Sarcoma-associated Herpesvirus (KSHV), is the essential causative agent. Co-infection with **EBV** is also present in about 60-70% of cases. * **Option D (True):** PEL is most commonly seen in patients with advanced **HIV/AIDS** (low CD4 counts) or other profound states of immunosuppression. ### High-Yield Clinical Pearls for NEET-PG: * **Immunophenotype:** Typically "null" phenotype (negative for CD19, CD20, CD79a) but positive for **CD45** and activation markers like **CD138** (syndecan-1), suggesting a plasma cell-like differentiation. * **Key Association:** Always look for the triad of **HIV + HHV-8 + Serous Effusion** in clinical vignettes. * **Prognosis:** Extremely poor; standard CHOP chemotherapy often has a limited response. * **Differential:** Do not confuse with *Burkitt Lymphoma* (c-myc translocation) or *Primary CNS Lymphoma* (EBV associated), both of which are also common in HIV patients [1].

Q: Burkitt's Lymphoma commonly affects which anatomical site?

Jaw. **Explanation:** Burkitt’s Lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma characterized by the translocation of the **c-MYC gene** (typically t(8;14)). The anatomical presentation of BL varies significantly based on its clinical variant [1]: 1. **Endemic (African) Variant:** This is the classic form strongly associated with **Epstein-Barr Virus (EBV)**. It characteristically presents as a rapidly growing mass in the **mandible or maxilla (jaw)** in children [1]. This makes **Option A** the most common site associated with the classic description of the disease. 2. **Sporadic (Non-African) Variant:** This form is less associated with EBV and typically presents as an **abdominal mass**, most commonly involving the **ileocecal region** [1]. **Analysis of Incorrect Options:** * **B. Stomach:** While the sporadic form involves the abdomen, it primarily affects the distal ileum and cecum rather than the stomach (which is more common for MALTomas). * **C. Spleen:** Splenomegaly can occur, but the spleen is rarely the primary or defining site of presentation for BL. * **D. Kidneys:** Renal involvement can occur as part of systemic spread or in the sporadic form, but it is not the "classic" anatomical site used to identify the disease in exams [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Morphology:** Characterized by a **"Starry Sky" appearance** (tingible body macrophages representing the "stars" against a background of dark neoplastic B-cells). * **Immunophenotype:** Positive for CD19, CD20, CD10, and **BCL-6**; notably **BCL-2 negative**. * **Proliferation:** It has one of the highest proliferation rates (Ki-67 index often approaching **100%**). * **Genetics:** t(8;14) is most common; variants include t(2;8) and t(8;22). **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. 605-606.

Q: Auer rods are seen in which of the following cells?

Myeloblast. **Explanation:** **Auer rods** are pathognomonic morphological features of the **myeloid lineage** [1]. They are elongated, needle-like, pink or red-staining cytoplasmic inclusions formed by the fusion and crystallization of primary (azurophilic) granules [2]. 1. **Why Myeloblasts are correct:** Auer rods contain peroxidase, lysosomal enzymes, and crystalloid mucopolysaccharides. Since these granules are specific to the granulocytic series, Auer rods are found in **myeloblasts** and occasionally in promyelocytes (especially in APL) [1], [2]. Their presence definitively excludes a diagnosis of Acute Lymphoblastic Leukemia (ALL). 2. **Why other options are incorrect:** * **Lymphoblasts:** These cells belong to the lymphoid lineage, which lacks azurophilic granules and peroxidase; therefore, they never contain Auer rods [2]. * **Erythroblasts & Megakaryoblasts:** While these are part of the myeloid family (non-lymphoid), they do not typically form these specific crystalline inclusions. Auer rods are markers of **granulocytic differentiation** [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Acute Promyelocytic Leukemia (AML-M3):** Characterized by "faggot cells," which contain bundles or clusters of numerous Auer rods [2]. * **Staining:** Auer rods are strongly **Myeloperoxidase (MPO)** positive and **Sudan Black B** positive. * **Diagnostic Significance:** If you see an Auer rod, the diagnosis is **AML** (Acute Myeloid Leukemia) until proven otherwise [1]. They are never seen in Chronic Myeloid Leukemia (CML) unless it has entered a "myeloid blast crisis." **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. [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. 621-622.

Q: Which of the following conditions is not transmitted familially?

Factor V Leiden mutation. **Explanation:** The correct answer is **Factor V Leiden mutation**. While this condition is a genetic disorder, the question asks which is not "transmitted familially" in the context of classical Mendelian inheritance patterns typically seen in the other options. 1. **Factor V Leiden Mutation:** This is an autosomal dominant condition caused by a point mutation (G1691A) in the Factor V gene. While it is inherited, it is often categorized as a **thrombophilia** where environmental triggers (surgery, pregnancy, oral contraceptives) play a massive role in clinical expression. In many medical examinations, it is contrasted against classical hematologic "diseases" because it is a genetic risk factor rather than a guaranteed clinical disease state. *Note: If this question appears in a competitive exam, it is often a "best fit" answer or potentially a controversial recall, as all four options technically have genetic bases.* 2. **Thalassemia:** This is a classic **autosomal recessive** hemoglobinopathy caused by deficient synthesis of alpha or beta globin chains. It follows strict familial transmission patterns [1]. 3. **Hemophilia:** Hemophilia A and B are classic **X-linked recessive** disorders. They show a clear familial pattern, typically affecting males while females act as carriers. 4. **Sickle Cell Anemia:** This is an **autosomal recessive** disorder caused by a point mutation in the ̢-globin chain (glutamic acid replaced by valine) [1]. It is strictly transmitted from parents to offspring. **Clinical Pearls for NEET-PG:** * **Factor V Leiden** is the **most common** inherited cause of hypercoagulability (thrombophilia) in Caucasians. * The mutation makes Factor V resistant to inactivation by **Activated Protein C (APC)**. * **High-yield inheritance:** Hemophilia (X-linked), Thalassemia/Sickle Cell (Autosomal Recessive), Hereditary Spherocytosis (Autosomal Dominant) [2].

Question 1

Cold agglutinins are directly against which of the following RBC antigens?

Accepted Answer

I antigen

Answer

P antigen

Answer

Le antigen

Answer

Rh antigen

Question 2

A 20-year-old female presents with a 5-day history of fatigue, low-grade fever, and sore throat. Physical examination reveals bilateral enlarged, tender cervical lymph nodes, an exudative tonsillitis, and an enlarged spleen. A complete blood cell count reveals the hemoglobin and platelet counts to be within normal limits. The total white blood cell count is increased to 9200 cells per uL. Examination of the peripheral blood reveals the presence of atypical mononuclear cells with abundant cytoplasm. These cells have peripheral condensation of the cytoplasm, which gives them a "ballerina ski" appearance. Which one of the listed findings is most likely to be present in this individual?

Accepted Answer

Heterophil antibodies in the serum

Answer

Aggregates of mononuclear cells with cytoplasmic Birbeck granules in the liver

Answer

Elevated levels of d-ALA in the urine

Answer

Group A streptococcus on culture of the tonsillar exudate

Question 3

A 70-year-old male has been experiencing intermittent epistaxis, fatigue, and bone pain for the past 4 months. Laboratory investigations reveal serum calcium of 12 mg/dL. What is the most likely finding in a bone marrow aspirate from this patient?

Accepted Answer

Numerous plasma cells

Answer

Sheets of myeloid blast cells

Answer

A 'dry' tap

Answer

Abundance of lymphoid blast cells

Question 4

All of the following statements regarding primary effusion lymphoma are true EXCEPT?

Accepted Answer

The proliferating cells are NK cells

Answer

Also referred to as body cavity lymphoma

Answer

There is often an association with HHV-8

Answer

Patients are commonly HIV positive

Question 5

Burkitt's Lymphoma commonly affects which anatomical site?

Accepted Answer

Jaw

Answer

Stomach

Answer

Spleen

Answer

Kidneys

Question 6

In which condition is Thrombin time raised?

Accepted Answer

Afibrinogenemia

Answer

Hemophilia

Answer

Factor XII deficiency

Answer

von Willebrand's disease

Question 7

Auer rods are seen in which of the following cells?

Accepted Answer

Myeloblast

Answer

Lymphoblast

Answer

Erythroblast

Answer

Megakaryoblast

Question 8

What are the diagnostic criteria for Hodgkin's disease?

Accepted Answer

Atypical cells in a specific background milieu

Answer

Reed-Sternberg (RS) cells

Answer

Presence of a sclerosing pattern

Answer

CD30 positivity

Question 9

All of the following are true regarding Leukemoid reaction, EXCEPT:

Accepted Answer

Neutrophil count >= 11600/mL

Answer

Blast cells <5%

Answer

Platelet count 100,000/mL

Answer

Hemoglobin > 9 gm%

Question 10

Which of the following conditions is not transmitted familially?

Accepted Answer

Factor V Leiden mutation

Answer

Thalassemia

Answer

Hemophilia

Answer

Sickle cell anemia

Hematopathology — MCQs

Hematopathology — MCQs

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