White Blood Cell Disorders — MCQs
An 80-year-old asymptomatic man presents with a total leukocyte count of 100,000, with 80% lymphocytes and 20% PMCs. What is the most probable diagnosis?
Which of the following tests would be most beneficial in the diagnosis of a patient suspected of having chronic granulomatous disease of childhood?
An 18-year-old male presents to the OPD with gum bleeding, fever, low total leukocyte count (TLC), and low platelet count. General examination is unremarkable. Further investigations reveal a low reticulocyte count, absent megakaryocytes, and no immature cells in the bone marrow. What is the most likely diagnosis?
Eosinophilic meningitis is seen with all except:
A baby presents with recurrent ear infections with discharge, seborrheic dermatitis, hepatosplenomegaly, and cystic skull lesions. What is the most likely diagnosis?
Tropical pulmonary eosinophilia is most characteristically seen due to which of the following infections?
Haemoglobin F is raised in which condition?
What is a known complication of Parvovirus B19 infection?
Which of the following is a characteristic bone marrow finding in myelofibrosis?
Splenectomy is least useful in
White Blood Cell Disorders Indian Medical PG Practice Questions and MCQs
Question 1: An 80-year-old asymptomatic man presents with a total leukocyte count of 100,000, with 80% lymphocytes and 20% PMCs. What is the most probable diagnosis?
- A. CML
- B. CLL (Correct Answer)
- C. TB
- D. HIV
Explanation: CLL - The finding of a **very high total leukocyte count** predominantly composed of **lymphocytes** (80%), particularly in an **asymptomatic elderly man**, is highly characteristic of **Chronic Lymphocytic Leukemia (CLL)** [1]. - CLL is often diagnosed incidentally in older patients due to routine blood tests showing **marked lymphocytosis** [1]. CML - **Chronic Myeloid Leukemia (CML)** would typically present with a high leukocyte count primarily consisting of **granulocytes** (neutrophils, eosinophils, basophils), and their precursors, not lymphocytes [2]. - CML is usually associated with the **Philadelphia chromosome (BCR-ABL1 fusion gene)** and often causes symptoms like fatigue, weight loss, and splenomegaly [2]. TB - **Tuberculosis (TB)** can cause a change in leukocyte count, but it typically presents with a **mild to moderate leukocytosis** with a **neutrophilic predominance** or **monocytosis**, and can also cause **lymphopenia** in severe cases, not the marked lymphocytosis seen here [1]. - Patients with TB are usually **symptomatic** with fever, night sweats, cough, and weight loss [3]. HIV - **HIV infection** primarily targets **CD4+ T-lymphocytes**, leading to a progressive decline in their count, resulting in **lymphopenia**, not the extreme lymphocytosis observed in this case [1]. - While HIV can cause various hematological abnormalities, significant, sustained lymphocytosis is not a typical hallmark [1].
Question 2: Which of the following tests would be most beneficial in the diagnosis of a patient suspected of having chronic granulomatous disease of childhood?
- A. E rosette - forming assay
- B. Cell-mediated cytolysis
- C. Nitroblue tetrazolium (NBT) test (Correct Answer)
- D. Determination of CD4: CD8 ratio
Explanation: ***Nitroblue tetrazolium (NBT) test*** - The **NBT test** is the **specific diagnostic test** for **chronic granulomatous disease (CGD)** as it directly assesses the defective pathophysiology. - CGD results from defective **NADPH oxidase**, preventing phagocytes from producing a **respiratory burst** needed to kill intracellular pathogens. - In the NBT test, normal phagocytes reduce the yellow NBT dye to blue **formazan** via superoxide production. In CGD, this reaction **fails**, confirming the diagnosis. - This functional assay directly demonstrates the **oxidative burst defect** characteristic of CGD. *E rosette - forming assay* - This assay identifies **T lymphocytes** by their ability to bind sheep red blood cells. - It evaluates **cellular immunity** and T cell numbers, not phagocyte function. - CGD is a **phagocytic disorder**, not a T cell deficiency, making this test irrelevant for diagnosis. *Cell-mediated cytolysis* - This test measures the killing ability of **cytotoxic T lymphocytes (CTLs)** and **NK cells** against target cells. - It assesses **adaptive and innate cellular immunity** but does not evaluate the **phagocytic oxidative burst** that is defective in CGD. *Determination of CD4: CD8 ratio* - This ratio evaluates the balance between **helper (CD4+)** and **cytotoxic (CD8+)** T cell subsets. - It is useful for diagnosing **HIV infection, autoimmune diseases**, and monitoring immunosuppression. - It does not assess **phagocyte function** and is not relevant to the diagnosis of CGD.
Question 3: An 18-year-old male presents to the OPD with gum bleeding, fever, low total leukocyte count (TLC), and low platelet count. General examination is unremarkable. Further investigations reveal a low reticulocyte count, absent megakaryocytes, and no immature cells in the bone marrow. What is the most likely diagnosis?
- A. Immune Thrombocytopenic Purpura (ITP)
- B. Myelodysplastic Syndrome (MDS)
- C. Aplastic anemia (Correct Answer)
- D. Acute Myeloid Leukemia (AML)
Explanation: ***Aplastic anemia*** - This condition is characterized by **pancytopenia** (low TLC and platelet count, implied low red blood cell count by low reticulocyte count) due to **bone marrow failure**. - The absence of **megakaryocytes** and other immature cells in the bone marrow confirms the **hypocellularity** typical of aplastic anemia. *Immune Thrombocytopenic Purpura (ITP)* - While ITP presents with **low platelet count** and potential gum bleeding [1], the bone marrow typically shows **normal or increased megakaryocytes**. - ITP does not explain the **low total leukocyte count** or low reticulocyte count seen in this patient [1]. *Myelodysplastic Syndrome (MDS)* - MDS involves **ineffective hematopoiesis** and can present with cytopenias, but the bone marrow is usually **hypercellular or normocellular** with dysplastic changes. - The absence of immature cells and overall hypoplasia does not fit the typical picture of MDS. *Acute Myeloid Leukemia (AML)* - AML is characterized by an overproduction of **immature myeloid cells (blasts)** [3], which are conspicuously absent in this patient's bone marrow description. - While AML can cause pancytopenia, the presence of **immature cells** in the bone marrow is its hallmark [3]. Gum hypertrophy is also a common clinical sign in AML [2].
Question 4: Eosinophilic meningitis is seen with all except:
- A. Cryptococcal meningitis (Correct Answer)
- B. Coccidioidomycosis
- C. Helminthic infections
- D. Leptomeningeal metastasis
Explanation: ***Cryptococcal meningitis*** - This is a **fungal infection** that typically causes a **lymphocytic or monocytic pleocytosis** in the cerebrospinal fluid (CSF), not eosinophilic meningitis. - While it can occur in immunocompromised individuals, **eosinophilia in the CSF** is not a characteristic feature. *Coccidioidomycosis* - This **fungal infection** can cause meningitis with a notable **eosinophilic pleocytosis** in the CSF [1]. - It is known to produce a chronic meningitis that may present with CSF eosinophilia. *Helminthic infections* - **Parasitic infections** like **Strongyloides, Gnathostoma, and Angiostrongylus cantonensis** are well-known causes of eosinophilic meningitis [2]. - The host immune response to these parasites often involves a strong **eosinophilic infiltrate** [2]. *Leptomeningeal metastasis* - **Leptomeningeal carcinomatosis** (metastasis) can cause a variety of CSF findings, including an **eosinophilic pleocytosis** [3]. - This is particularly true for certain types of cancers like **lymphoma** or **small cell lung cancer**.
Question 5: A baby presents with recurrent ear infections with discharge, seborrheic dermatitis, hepatosplenomegaly, and cystic skull lesions. What is the most likely diagnosis?
- A. Hemophagocytic lymphohistiocytosis
- B. Multiple myeloma
- C. Langerhans cell histiocytosis (Correct Answer)
- D. Acute lymphoblastic leukemia (ALL)
Explanation: ***Langerhans cell histiocytosis*** - This classic presentation involves a constellation of symptoms including **seborrheic dermatitis-like rash**, recurrent ear infections with discharge (due to infiltration of the mastoid bone), hepatosplenomegaly, and **cystic skull lesions**, all highly suggestive of Langerhans cell histiocytosis (LCH). - LCH is a rare disorder characterized by the proliferation of abnormal Langerhans cells, which can affect various organs and systems. *Hemophagocytic lymphohistiocytosis* - While patients with Hemophagocytic Lymphohistiocytosis (HLH) can present with **hepatosplenomegaly** and recurrent infections, they typically have high fevers, cytopenias, and poor clinical condition. - HLH does not typically cause **seborrheic dermatitis-like rashes** or **cystic bone lesions**. *Multiple myeloma* - Multiple myeloma is a **plasma cell malignancy** that predominantly affects older adults and is characterized by bone pain, anemia, renal failure, and hypercalcemia. - It does not present with recurrent ear infections, seborrheic dermatitis, or hepatosplenomegaly in this manner, and is exceedingly rare in infants. *Acute lymphoblastic leukemia (ALL)* - ALL is the most common childhood cancer and can cause **hepatosplenomegaly**, recurrent infections (due to neutropenia), and sometimes bone pain. - However, **seborrheic dermatitis-like rash** and **cystic skull lesions** are not typical features of ALL.
Question 6: Tropical pulmonary eosinophilia is most characteristically seen due to which of the following infections?
- A. Roundworm
- B. Trichinella
- C. Ancylostoma
- D. Filaria (Correct Answer)
Explanation: *Filaria* - **Tropical pulmonary eosinophilia (TPE)** is a hypersensitivity reaction to microfilariae from filarial nematodes like *Wuchereria bancrofti* and *Brugia malayi* [1]. - It is characterized by cough, dyspnea, wheezing, and marked **peripheral eosinophilia**, with interstitial infiltrates on chest X-ray [1]. *Roundworm* - **Ascaris lumbricoides** can cause **Loeffler's syndrome**, a transient pulmonary infiltration with eosinophilia during larval migration, but not chronic TPE [2]. - Symptoms are usually less severe and self-limiting compared to TPE [2]. *Trichinella* - **Trichinella spiralis** causes **trichinellosis**, presenting with muscle pain, fever, and periorbital edema, possibly with eosinophilia, but typically does not manifest as TPE. - Pulmonary involvement is rare and not the characteristic feature. *Ancylostoma* - **Hookworm (Ancylostoma duodenale, Necator americanus)** larvae can cause mild pulmonary symptoms and eosinophilia during migration through the lungs [3]. - However, they also do not typically lead to the severe and chronic pulmonary symptoms seen in TPE [3].
Question 7: Haemoglobin F is raised in which condition?
- A. Hereditary persistence of fetal hemoglobin (HPFH)
- B. Beta-thalassemia major
- C. Sickle cell disease
- D. Juvenile chronic myeloid leukemia (Correct Answer)
Explanation: ***Juvenile chronic myeloid leukemia*** - This condition is characterized by a high proportion of **fetal hemoglobin (HbF)**, often exceeding 50%, alongside other typical myeloproliferative features. - The elevated HbF is a distinguishing feature of **juvenile CML** from adult CML, which typically presents with normal or only slightly elevated HbF levels. *Beta-thalassemia major* - While patients with **beta-thalassemia major** can have elevated HbF, it is typically in response to a severe deficiency in beta-globin chain production, leading to compensatory gamma-chain synthesis. - However, the primary genetic defect lies in the beta-globin genes, and the HbF increase is usually not as universally high or definitive as in HPFH or juvenile CML. *Sickle cell disease* - Patients with **sickle cell disease** can have variable levels of HbF, and higher levels are associated with a milder disease course [1]. - HbF acts as a protective factor by inhibiting hemoglobin S polymerization, but the presence of high HbF is not a diagnostic marker in the same way it is for HPFH or juvenile CML [1]. *Hereditary persistence of fetal hemoglobin (HPFH)* - This is a benign condition characterized by the **continued production of high levels of HbF into adulthood** due to genetic mutations that prevent the normal developmental switch from gamma-globin to beta-globin synthesis. - While it features significantly raised HbF, HPFH is typically **asymptomatic** and does not present with the myeloproliferative features seen in juvenile CML.
Question 8: What is a known complication of Parvovirus B19 infection?
- A. Erythema infectiosum
- B. Arthritis
- C. Aplastic anemia (Correct Answer)
- D. All of the options
Explanation: Aplastic anemia - Parvovirus B19 has a tropism for erythroid progenitor cells in the bone marrow, specifically targeting and destroying them [1]. - This destruction can lead to a transient aplastic crisis, especially in individuals with pre-existing hemolytic conditions, causing a severe drop in red blood cell production [1]. Erythema infectiosum - This is the most common clinical manifestation of Parvovirus B19 infection, also known as fifth disease, characterized by a "slapped cheek" rash [1]. - While it is a symptom or disease caused by the virus, it is not considered a complication in the sense of a secondary, adverse outcome. Arthritis - Arthralgia and arthritis are common manifestations of Parvovirus B19 infection, particularly in adults, especially women [1]. - Similar to erythema infectiosum, it is a direct clinical manifestation rather than a "complication" representing a secondary, undesirable event. All of the options - While erythema infectiosum and arthritis are common clinical presentations of Parvovirus B19, they are direct disease manifestations. - Aplastic anemia stands out as a true complication, representing a secondary and potentially severe adverse outcome due to the virus's specific cellular tropism [1].
Question 9: Which of the following is a characteristic bone marrow finding in myelofibrosis?
- A. Increased reticulin fibrosis (Correct Answer)
- B. Absence of fibroblasts
- C. Decreased megakaryocytes
- D. Hypocellular marrow from onset
Explanation: ***Leucoerythroblastosis*** - A hallmark finding in myelofibrosis is **leucoerythroblastosis**, characterized by the presence of immature white cells and nucleated red blood cells in the bloodstream [1][3]. - This reflects an **extramedullary hematopoiesis** due to the failure of normal marrow function and is commonly seen in myelofibrosis [1][2]. *Tear drop cells* - While **tear drop cells** (dacryocytes) can be associated with myelofibrosis, they are not exclusive findings and can appear in other conditions [1]. - They are indicative of **extramedullary hematopoiesis** but are not definitive for myelofibrosis specifically. *Leucocytopenia* - Myelofibrosis is associated with **neutrophilia** rather than leucocytopenia, which presents as low white blood cell counts. - The condition often leads to an increase in white blood cell counts due to reactive changes rather than a decrease. *All of the above* - This option is incorrect as it suggests that all previously mentioned findings are present in myelofibrosis. - Both **tear drop cells** and **leucocytopenia** are not definitive or accurate findings compared to **leucoerythroblastosis**. **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. 628-629. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 615-616. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Heart, pp. 589-590.
Question 10: Splenectomy is least useful in
- A. Hereditary nonspherocytic haemolytic anaemia (Correct Answer)
- B. Congenital elliptocytosis
- C. Thalassemia major
- D. Congenital spherocytic anaemia
Explanation: ***Hereditary nonspherocytic hemolytic anemia*** - This group of anemias primarily involves **defects in red blood cell enzymes**, such as G6PD deficiency or pyruvate kinase deficiency, leading to premature destruction of red blood cells (hemolysis) mainly in the **bone marrow** and peripheral circulation, not predominantly in the spleen. - Since the spleen is not the primary site of red blood cell destruction in these conditions, **splenectomy generally offers little to no benefit** and may even be contraindicated due to increased risks without significant clinical improvement. *Congenital elliptocytosis* - This condition involves **abnormalities in red blood cell membrane proteins**, leading to elliptical-shaped red blood cells that are more fragile and prone to splenic sequestration and destruction [1]. - **Splenectomy is a treatment option** for severe cases of congenital elliptocytosis, as it reduces splenic trapping and destruction of abnormal red blood cells, thereby alleviating anemia and hemolysis [1]. *Thalassemia major* - **Thalassemia major** is characterized by severe impairment of hemoglobin synthesis, leading to ineffective erythropoiesis, chronic hemolysis, and significant splenomegaly due to increased splenic sequestration and destruction of abnormal red blood cells. - **Splenectomy is often considered in thalassemia major** to reduce transfusion requirements and alleviate symptoms related to splenomegaly like abdominal discomfort and early satiety. *Congenital spherocytic anemia* - Also known as **hereditary spherocytosis**, this condition is caused by defects in red blood cell membrane proteins, leading to fragile, spherical red blood cells that are preferentially targeted and destroyed by the spleen [1]. - **Splenectomy is a highly effective treatment** for hereditary spherocytosis, as it removes the primary organ responsible for destroying the abnormal red blood cells, leading to a significant reduction in hemolysis and improvement in anemia [1].
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