HEMA-ONCO LE5 Flashcards
56. The treatment of choice for most cases of Type 1 von Willebrand disease is:
A. Desmopressin
B. von Willebrand factor (vWF) concentrate
C. Cryoprecipitate
D. Fresh frozen plasma
A. Desmopressin
Rationale:
💉 Desmopressin (DDAVP) is the treatment of choice for most cases of Type 1 von Willebrand disease (vWD), as it stimulates the release of stored vWF from endothelial cells.
🩸 vWF concentrate (B) is reserved for severe cases or surgery-related bleeding.
❌ Cryoprecipitate (C) and Fresh frozen plasma (D) are not preferred due to infection risk and inconsistent vWF levels.
57. In disseminated intravascular coagulation (DIC), which of the following is reduced?
A. Prothrombin time (PT)
B. Partial thromboplastin time (PTT)
C. D-dimer
D. Fibrinogen
E. Bleeding time
D. Fibrinogen
Rationale:
⚠️ DIC is a consumptive coagulopathy leading to depletion of clotting factors, platelets, and fibrinogen.
📉 Fibrinogen levels are reduced due to excessive fibrin formation and breakdown.
📈 D-dimer is increased due to fibrinolysis.
⏳ PT and PTT are prolonged due to clotting factor consumption.
🩸 Bleeding time is increased but is not a specific marker for DIC.
58. All of the following are treatment options for immune thrombocytopenia (ITP) EXCEPT:
A. Steroids
B. Androgens
C. Anti-D immunoglobulin
D. Intravenous immunoglobulin (IVIG)
E. Splenectomy
B. Androgens
Rationale:
🩺 Immune thrombocytopenia (ITP) is managed with:
Steroids (A) to suppress the immune response.
Anti-D immunoglobulin (C) for Rh+ patients.
IVIG (D) to rapidly increase platelet count.
Splenectomy (E) in refractory cases.
🚫 Androgens (e.g., danazol) are not used in ITP, but they may be used in other hematologic disorders like aplastic anemia.
59. A 2-year-old boy presents with recurrent skin and respiratory tract infections since early infancy. He has eczematous skin on the upper and lower limbs. Initial laboratory tests show normal liver and renal functions, but a complete blood count (CBC) reveals thrombocytopenia. He has a similarly affected male cousin. The most likely diagnosis is:
A. Glanzmann thrombasthenia
B. Wiskott-Aldrich syndrome
C. Bernard-Soulier syndrome
D. Immune thrombocytopenia
B. Wiskott-Aldrich syndrome
Rationale:
🧬 Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder with the classic triad:
Thrombocytopenia (small platelets)
Eczema
Recurrent infections due to immunodeficiency
👦 More common in boys, and a family history of affected males supports the diagnosis.
🛑 Glanzmann thrombasthenia (A) and Bernard-Soulier syndrome (C) involve platelet dysfunction but not immunodeficiency or eczema.
🔬 Immune thrombocytopenia (D) does not cause eczema or immunodeficiency.
60. A 5-year-old boy presents to the emergency room with sudden onset of skin ecchymoses and a single episode of epistaxis. His history is unremarkable except for a preceding upper respiratory tract infection (URTI). On examination, he appears active and healthy, with a diffuse petechial rash and ecchymoses on the upper and lower limbs. There is no lymphadenopathy or hepatosplenomegaly. His initial CBC results are as follows: Hb 12.3 g/dL, WBC 7,600/mm³ (Neutrophils 45%, Lymphocytes 51%), and platelet count 17,000/mm³. The peripheral blood smear shows rarely seen platelets. The most likely diagnosis is:
A. Immune thrombocytopenia
B. Acute leukemia
C. Hemophilia A
D. von Willebrand disease
A. Immune thrombocytopenia (ITP)
Rationale:
🤧 ITP is the most common cause of acute thrombocytopenia in children, often following a viral infection.
🩸 Platelet count < 100,000/mm³ with an otherwise normal CBC and peripheral smear (few platelets seen).
🚫 No hepatosplenomegaly, lymphadenopathy, or systemic symptoms, ruling out acute leukemia (B).
🧬 Hemophilia A (C) and von Willebrand disease (D) cause prolonged bleeding but not isolated thrombocytopenia.
61. Which of the following symptoms or signs is a manifestation of anemia?
A. Irritability
B. Pharyngitis
C. Bruising
D. Fatigue
D. Fatigue
Rationale:
🩸 Anemia leads to reduced oxygen delivery to tissues, causing fatigue as a common symptom.
😠 Irritability (A) can be seen in severe anemia but is not a primary symptom.
🦠 Pharyngitis (B) is related to infections, not anemia.
🔵 Bruising (C) is more indicative of platelet disorders or coagulopathies.
62. Which of the following statements about the adaptive physiologic response in anemia is inaccurate?
A. There is a shift to the left in the oxygen dissociation curve.
B. An increase in erythropoietin leads to increased RBC production.
C. There is a shift to the right in the oxygen dissociation curve.
D. There is an observed physiologic expansion of intravascular volume.
A. There is a shift to the left in the oxygen dissociation curve.
Rationale:
🫁 In anemia, tissues receive less oxygen, triggering a shift to the right in the oxygen dissociation curve (C).
🩸 Increased erythropoietin (B) stimulates RBC production as a compensatory response.
💧 Physiologic expansion of intravascular volume (D) occurs to maintain circulation.
🚫 A left shift (A) would mean hemoglobin holds onto oxygen more tightly, reducing oxygen delivery, which is not adaptive in anemia.
63. The most common cause of iron deficiency among Filipino children is:
A. Hookworm infestation
B. Prematurity
C. Inadequate dietary intake
D. Menstrual losses
C. Inadequate dietary intake
Rationale:
🍽️ Iron deficiency anemia (IDA) in Filipino children is primarily due to inadequate dietary intake of iron-rich foods.
🐛 Hookworm infestation (A) can contribute but is not the leading cause.
👶 Prematurity (B) increases risk due to low iron stores but is less common than dietary deficiency.
🩸 Menstrual losses (D) are relevant in adolescents, not young children.
64. Which of the following symptoms or signs is a manifestation of polycythemia?
A. Lymphadenopathy
B. Deep vein thrombosis
C. Epistaxis
D. Seizures
D. Seizures
Rationale:
🩸 Polycythemia (excess RBCs) increases blood viscosity, reducing oxygen delivery to the brain, which can trigger seizures.
🦠 Lymphadenopathy (A) is seen in infections and malignancies, not polycythemia.
🩹 Deep vein thrombosis (B) and epistaxis (C) occur due to hypercoagulability but are not as definitive as seizures.
65. The urgency for packed red blood cell (PRBC) transfusion in childhood anemia should be dictated by:
A. Absolute level of hemoglobin
B. Extent of functional impairment
C. Age of the patient
D. Presence or absence of splenomegaly
B. Extent of functional impairment
Rationale:
🔬 The urgency of PRBC transfusion depends more on clinical symptoms than absolute hemoglobin levels.
🩸 Severe anemia with cardiovascular compromise (tachycardia, dyspnea) requires urgent transfusion.
📏 Absolute hemoglobin level (A) alone is not the best criterion.
👶 Age of the patient (C) and splenomegaly (D) do not dictate transfusion urgency.
66. A 13-month-old girl presents with progressive pallor for the last three months. The mother noticed that she started eating non-nutritive and dirty substances. She has been exclusively breastfed up to this age. On physical examination, she is pale and apathetic-looking, with no lymphadenopathy, organomegaly, or skin bleeding. The most likely diagnosis is:
A. Iron deficiency anemia
B. Folic acid deficiency
C. Vitamin B12 deficiency
D. Aplastic anemia
A. Iron deficiency anemia
Rationale:
🔴 Iron deficiency anemia (IDA) is common in exclusively breastfed infants beyond 6 months without iron supplementation.
🧃 Pica (eating non-nutritive substances) is a classic symptom of IDA.
🧬 Folic acid (B) and vitamin B12 deficiency (C) present with macrocytic anemia and neurologic symptoms.
🛑 Aplastic anemia (D) causes pancytopenia, not just isolated anemia.
67. A 7-year-old apparently healthy boy presents to the outpatient clinic with mild pallor and slight yellowish discoloration of the sclera since early childhood. On physical examination, he has a palpable spleen along with pallor and jaundice. His uncle had a history of repeated blood transfusions during childhood, which were relieved after splenectomy. The most likely diagnosis at this stage is:
A. Autoimmune hemolytic anemia
B. Hereditary spherocytosis
C. Acute leukemia
D. Hemolytic uremic syndrome
B. Hereditary spherocytosis
Rationale:
🧬 Hereditary spherocytosis (HS) presents with pallor, jaundice, and splenomegaly due to chronic hemolysis.
⚪ Spherocytes on peripheral smear confirm the diagnosis.
🩸 Family history of splenectomy improving symptoms supports HS.
🦠 Autoimmune hemolytic anemia (A) typically has a positive Coombs test.
🛑 Acute leukemia (C) and hemolytic uremic syndrome (D) present with systemic illness and thrombocytopenia, which are absent here.
68. Iron deficiency anemia is less prevalent in breastfed infants because:
A. Breast milk contains a higher amount of iron than cow’s milk.
B. The mother’s iron status is sufficient for the infant in the first year of life.
C. Iron in human milk is more efficiently absorbed.
D. Fortification of cow’s milk formula is inadequate.
C. Iron in human milk is more efficiently absorbed.
Rationale:
👶 Breastfed infants have lower iron deficiency rates because human milk contains iron in a highly bioavailable form (~50% absorption).
🐄 Cow’s milk (A) has more iron but much lower absorption (~10%).
🩸 Maternal iron stores (B) support the infant for only the first 4-6 months.
🍼 Iron-fortified formula (D) is beneficial but does not apply to exclusively breastfed infants.
69. An 8-year-old boy presents with recurrent skin ecchymoses for the past few months. On physical examination, he has café-au-lait spots, microcephaly, and an absent thumb. The most likely diagnosis is:
A. Neurofibromatosis
B. Fanconi anemia
C. Peutz-Jeghers syndrome
D. Sickle cell anemia
B. Fanconi anemia
Rationale:
🧬 Fanconi anemia is a genetic bone marrow failure syndrome associated with:
Café-au-lait spots
Microcephaly and absent thumbs
Pancytopenia and increased leukemia risk
🛑 Neurofibromatosis (A) has café-au-lait spots but no hematologic abnormalities.
⚠️ Peutz-Jeghers syndrome (C) presents with GI polyps and mucocutaneous pigmentation.
🔴 Sickle cell anemia (D) causes vaso-occlusive crises, not bone marrow failure.
70. Splenectomy in acute immune thrombocytopenia (ITP) is indicated:
A. After failure of steroid therapy
B. Concomitant with immunoglobulin therapy
C. In life-threatening bleeding
D. To reduce the rate of platelet transfusions
C. In life-threatening bleeding
Rationale:
🩺 Splenectomy is reserved for severe, refractory ITP cases, particularly those with life-threatening bleeding.
💊 Steroids (A) and IVIG (B) are first-line treatments.
🩸 Splenectomy is not done simply to reduce platelet transfusions (D).
71. Which hemoglobin type is markedly elevated in Beta thalassemia major?
A. Hb A1
B. Hb F
C. Hb A2
D. Hb H
B. Hb F
Rationale:
🩸 Beta thalassemia major is characterized by absent or severely reduced Hb A1 production, leading to compensatory elevation of fetal hemoglobin (Hb F).
🚼 Hb F remains the dominant hemoglobin type in affected individuals.
❌ Hb A2 (C) is mildly increased in β-thalassemia minor but not in major.
❌ Hb H (D) is seen in α-thalassemia, not β-thalassemia.
72. Why should an ultrasound of the hepatobiliary tree be requested in a child with hereditary spherocytosis before splenectomy?
A. To rule out gallstones
B. To rule out cholecystitis
C. To rule out hepatitis
D. To check the patency of the common bile duct
A. To rule out gallstones
Rationale:
🫁 Hereditary spherocytosis (HS) leads to chronic hemolysis, increasing bilirubin turnover and predisposing to gallstone formation.
🔬 Ultrasound of the hepatobiliary tree is necessary before splenectomy to check for pigment gallstones.
🚫 Cholecystitis (B) and hepatitis (C) are not primary concerns in HS.
🚫 Bile duct patency (D) is not a primary indication for imaging in HS.
73. A common significant feature of a blood smear in lead poisoning is:
A. Normochromic RBC
B. Macrocytic RBC
C. Basophilic stippling
D. Target cells
C. Basophilic stippling
Rationale:
🔬 Lead poisoning disrupts hemoglobin synthesis, causing ribosomal aggregation, visible as basophilic stippling on peripheral blood smear.
🚫 Normochromic (A) and macrocytic RBCs (B) are not characteristic.
🚫 Target cells (D) are seen in thalassemia, not lead poisoning.
74. A key feature that signifies β-thalassemia minor is:
A. Low iron level
B. Absent marrow sideroblasts
C. Elevated hemoglobin A2
D. High red cell distribution width
C. Elevated hemoglobin A2
Rationale:
🩸 β-thalassemia minor is characterized by a mild microcytic anemia with elevated Hb A2 (>3.5%) on hemoglobin electrophoresis.
🚫 Iron levels (A) are typically normal.
🚫 Marrow sideroblasts (B) are seen in sideroblastic anemia, not thalassemia.
🚫 Red cell distribution width (D) is usually normal in β-thalassemia minor.
75. Which of the following hemoglobinopathies is transfusion-dependent?
A. α-thalassemia trait
B. Heterozygous β-thalassemia
C. Hemoglobin H disease
D. Homozygous β-thalassemia
D. Homozygous β-thalassemia
Rationale:
🩸 Homozygous β-thalassemia (β-thalassemia major) is transfusion-dependent due to severe anemia.
❌ α-thalassemia trait (A) and heterozygous β-thalassemia (B) are asymptomatic or mild.
🩸 Hemoglobin H disease (C) can be severe but is usually not transfusion-dependent.
76. Which of the following viruses can cause aplastic crises in chronic hemolytic disease?
A. Herpes virus
B. Human Parvovirus B19
C. Cytomegalovirus
D. T-lymphocyte virus
B. Human Parvovirus B19
Rationale:
🦠 Parvovirus B19 infects erythroid progenitor cells, causing transient red cell aplasia, leading to aplastic crises in chronic hemolytic diseases (e.g., sickle cell disease, hereditary spherocytosis).
🚫 Herpes virus (A), Cytomegalovirus (C), and T-lymphocyte virus (D) do not cause aplastic crises.
77. A 6-year-old girl presents with recurrent mucocutaneous bleeding episodes since early childhood, usually stopping spontaneously or with local measures. Her brother also has similar symptoms. Initial CBC is normal. The most likely diagnosis is:
A. Hemophilia A
B. Hemophilia B
C. von Willebrand disease
D. Disseminated intravascular coagulation
C. von Willebrand disease
Rationale:
🩸 von Willebrand disease (vWD) is the most common inherited bleeding disorder, characterized by mucocutaneous bleeding and normal CBC.
👨👩👧 Autosomal dominant inheritance explains affected siblings.
🚫 Hemophilia A (A) and Hemophilia B (B) cause deep joint/muscle bleeding.
🚫 DIC (D) presents with thrombocytopenia and prolonged coagulation times.
78. Which of the following criteria is included in the definition of severe aplastic anemia?
A. Reticulocyte count less than 1.0%
B. Neutrophil count less than 500/mm³
C. Platelet count less than 50,000/mm³
D. Bone marrow cellularity less than 50% of normal
B. Neutrophil count less than 500/mm³
Rationale:
🩸 Severe aplastic anemia is defined by bone marrow failure with profound pancytopenia, including:
Neutrophils <500/mm³
Platelets <20,000/mm³
Reticulocytes <1%
🔬 Bone marrow cellularity is <25%, not <50% (D).
79. The role of corticosteroids in autoimmune hemolytic anemia is to decrease:
A. Antibody production
B. The size of the spleen
C. The clearance of sensitized RBCs in the spleen
D. The immune response to offending organisms
C. The clearance of sensitized RBCs in the spleen
Rationale:
🩺 Corticosteroids decrease macrophage-mediated clearance of antibody-coated RBCs in autoimmune hemolytic anemia.
🚫 They do not directly reduce antibody production (A) or spleen size (B).
🚫 They do not target infectious organisms (D).
80. A common site for osteosarcoma is:
A. Distal humerus
B. Distal tibia
C. Distal femur
D. Proximal fibula
C. Distal femur
Rationale:
🦴 Osteosarcoma commonly affects the metaphysis of long bones, particularly the distal femur and proximal tibia.
📊 Most common sites:
Distal femur (42%)
Proximal tibia (19%)
Proximal humerus (10%)
Pelvis (8%)
🚫 Distal humerus (A) and proximal fibula (D) are rare sites.
81. The most common soft tissue sarcoma in children is:
A. Fibrosarcoma
B. Ewing sarcoma
C. Myofibroma
D. Rhabdomyosarcoma
D. Rhabdomyosarcoma
Rationale:
🦠 Rhabdomyosarcoma is the most common soft tissue sarcoma in children, typically arising from skeletal muscle.
🩸 Fibrosarcoma (A) and myofibroma (C) are rare in children.
🦴 Ewing sarcoma (B) is a bone tumor, not a soft tissue sarcoma.
82. Individuals with hereditary retinoblastoma have an increased risk for:
A. Rhabdomyosarcoma
B. Osteosarcoma
C. Acute leukemia
D. Neuroblastoma
B. Osteosarcoma
Rationale:
🧬 Hereditary retinoblastoma (RB1 gene mutation) is associated with an increased risk of osteosarcoma.
🦠 Rhabdomyosarcoma (A), acute leukemia (C), and neuroblastoma (D) are not strongly linked to hereditary retinoblastoma.
83. What is a possible differentiating symptom between osteosarcoma and Ewing sarcoma?
A. Pain in osteosarcoma
B. Swelling in Ewing sarcoma
C. Fever in osteosarcoma
D. Weight loss in Ewing sarcoma
D. Weight loss in Ewing Sarcoma
📝 Rationale:
Ewing Sarcoma is more likely to present with systemic symptoms, including fever, weight loss, and malaise, distinguishing it from Osteosarcoma, which is usually more localized. Weight loss is a key differentiating factor because Ewing Sarcoma is a small round blue cell tumor of neuroectodermal origin, making it more likely to cause constitutional symptoms.
Key Differences: Osteosarcoma vs. Ewing Sarcoma
Feature Osteosarcoma 🌟 Ewing Sarcoma 🌟
Primary Age Group Adolescents (10-20 years) Children & young adults (5-25 years)
Location Metaphysis of long bones (femur, tibia) Diaphysis of long bones (femur, pelvis, ribs)
Symptoms Bone pain, localized swelling Pain, swelling, fever, weight loss
X-ray Findings Sunburst appearance, Codman’s triangle **Onion skin periosteal
84. Definitive diagnosis for sarcomas often requires:
A. CT chest
B. Bone scan
C. Bone marrow biopsy
D. Tissue biopsy
D. Tissue biopsy
Rationale:
🔬 Definitive diagnosis of sarcomas requires a tissue biopsy to confirm malignancy and specific histological subtype.
📸 CT chest (A) and bone scan (B) help with staging but do not confirm the diagnosis.
🩸 Bone marrow biopsy (C) is used in hematologic malignancies, not sarcomas.
85. A 3-year-old girl presents with prolonged fever, progressive pallor, arthralgia, and skin ecchymoses. The most likely diagnosis is:
A. Brain tumor
B. Hepatoblastoma
C. Leukemia
D. Rhabdomyosarcoma
E. Wilms’ tumor
C. Leukemia
Rationale:
🩸 Leukemia presents with fever, progressive pallor, bone pain (arthralgia), and bruising due to bone marrow failure.
🧠 Brain tumors (A) cause neurological symptoms, not bone marrow suppression.
🩺 Wilms’ tumor (E) and hepatoblastoma (B) typically present with an abdominal mass.
🦠 Rhabdomyosarcoma (D) presents as a localized mass, not systemic symptoms.
86. Which type of childhood leukemia carries the worst prognosis?
A. Adult-type chronic myeloid leukemia (CML)
B. Juvenile chronic myeloid leukemia (CML)
C. Acute lymphoblastic leukemia (ALL) L1
D. Acute myeloid leukemia (AML) M1
B. Juvenile chronic myeloid leukemia (CML)
Rationale:
🧬 Juvenile CML has a poor prognosis compared to adult-type CML and ALL.
🩸 ALL (C) has a better prognosis, especially in younger children.
🦠 AML (D) has an intermediate prognosis but is not the worst.
87. A 5-year-old boy presents with an abdominal mass, hematuria, and hypertension. The most likely diagnosis is:
A. Wilms’ tumor
B. Neuroblastoma
C. Brain tumor
D. Rhabdomyosarcoma
E. Brain tumor
A. Wilms’ tumor
Rationale:
🩺 Wilms’ tumor (nephroblastoma) presents with an abdominal mass, hematuria, and hypertension due to renal involvement.
🦠 Neuroblastoma (B) also presents with an abdominal mass but commonly has systemic symptoms (e.g., fever, weight loss).
🧠 Brain tumors (C, E) cause neurological symptoms, not abdominal masses.
🦴 Rhabdomyosarcoma (D) presents as a localized soft tissue mass, not kidney-related symptoms.
88. What is the most common primary malignant bone tumor in children and adolescents?
A. Ewing sarcoma
B. Osteosarcoma
C. Osteochondroma
D. Osteoblastoma
B. Osteosarcoma
Rationale:
🦴 Osteosarcoma is the most common primary malignant bone tumor in children and adolescents, commonly affecting the distal femur and proximal tibia.
🩸 Ewing sarcoma (A) is the second most common but less frequent than osteosarcoma.
🚫 Osteochondroma (C) and osteoblastoma (D) are benign bone tumors.
89. The best screening radiographic study for a patient with suspicious cervical lymphadenopathy, fever, and weight loss is:
A. Chest X-ray
B. Chest MRI
C. Abdominal CT scan
D. Neck X-ray
A. Chest X-ray
Rationale:
📸 A chest X-ray is the best initial screening for lymphadenopathy in suspected lymphoma, looking for a mediastinal mass.
🧲 MRI (B) and CT (C) are more detailed but not first-line screening tools.
🚫 Neck X-ray (D) is not useful for lymphadenopathy assessment.
90. A 10-year-old girl with newly diagnosed abdominal lymphoma was admitted with difficulty breathing and poor urine output over the past day. Her biochemical renal indices show elevated urea and creatinine, with electrolyte disturbances including hyperkalemia, hyperuricemia, and hyperphosphatemia. The most likely diagnosis is:
A. Renal infiltration by lymphoma
B. Hemolytic uremic syndrome
C. Tumor lysis syndrome
D. Leukostasis
C. Tumor lysis syndrome
Rationale:
⚠️ Tumor lysis syndrome (TLS) occurs after chemotherapy or spontaneously in high-burden malignancies like lymphoma and leukemia, causing:
Hyperkalemia
Hyperphosphatemia
Hyperuricemia
Acute kidney injury (AKI)
🩸 Renal infiltration (A) does not cause electrolyte disturbances.
🦠 Hemolytic uremic syndrome (B) presents with thrombocytopenia and hemolysis.
🩺 Leukostasis (D) is seen in hyperleukocytosis but does not typically present with TLS-like findings.
91. Tumor lysis syndrome is most commonly associated with which of the following malignancies?
A. Neuroblastoma
B. Wilms’ tumor
C. Hepatoblastoma
D. Leukemia and Lymphoma
D. Leukemia and Lymphoma
Rationale:
⚠️ Tumor lysis syndrome (TLS) occurs in malignancies with high cell turnover and rapid response to therapy, such as acute leukemias and high-grade lymphomas (e.g., Burkitt lymphoma).
🚫 Neuroblastoma (A), Wilms’ tumor (B), and hepatoblastoma (C) are not typically associated with TLS.
92. The most common clinical presentation of Hodgkin disease in children is painless lymph node enlargement in which region?
A. Mediastinal
B. Cervical
C. Abdominal
D. Inguinal
E. Axillary
B. Cervical
Rationale:
🩺 The most common clinical presentation of Hodgkin lymphoma in children is painless cervical lymphadenopathy.
🫁 Mediastinal involvement (A) occurs but is usually seen in older children and adolescents.
🚫 Abdominal (C), inguinal (D), and axillary (E) lymph nodes are less commonly affected initially.
93. The most common site for metastasis of osteosarcoma is:
A. Kidneys
B. Bone marrow
C. Lungs
D. Liver
C. Lungs
Rationale:
🫁 The lungs are the most common site of metastasis for osteosarcoma due to hematogenous spread.
🚫 Bone marrow (B) and liver (D) are less commonly involved in metastasis.
🚫 Kidneys (A) are not a common metastatic site.
94. Rb gene analysis is useful in the workup of:
A. Wilms’ tumor
B. Neuroblastoma
C. Rhabdomyosarcoma
D. Retinoblastoma
D. Retinoblastoma
Rationale:
🧬 RB1 gene mutations cause retinoblastoma, a primary pediatric eye cancer.
🚫 Wilms’ tumor (A) is associated with WT1 gene mutations.
🚫 Neuroblastoma (B) is linked to MYCN amplification.
🚫 Rhabdomyosarcoma (C) does not involve RB1 mutations.
95. The most common solid tumors in children are:
A. Lymphomas
B. Sarcomas
C. Brain tumors
D. Renal tumors
C. Brain tumors
Rationale:
🧠 Brain tumors are the most common solid tumors in children, surpassing lymphomas and sarcomas.
🚫 Lymphomas (A) are hematologic malignancies, not solid tumors.
🚫 Renal tumors (D), including Wilms’ tumor, are less common than brain tumors.
96. The most common histopathological subtype of childhood Hodgkin disease is:
A. Lymphocyte predominant
B. Nodular sclerosis
C. Mixed cellularity
D. Lymphocyte depletion
E. Undifferentiated
B. Nodular sclerosis
Rationale:
🦠 Nodular sclerosis is the most common histologic subtype of Hodgkin lymphoma in children.
🧪 Mixed cellularity (C) is more common in developing countries.
🚫 Lymphocyte-predominant (A) and lymphocyte-depleted (D) subtypes are less common.
97. WAGR syndrome includes which of the following conditions?
A. Wilms tumor, aniridia, genitourinary malformation, and mental retardation
B. Wilms tumor, aniridia, gigantism, and renal failure
C. Wilms tumor, anisochromia, genitourinary malformation, and rickets
D. Wilms tumor, asplenia, genitourinary malformation, and rickets
A. Wilms tumor, aniridia, genitourinary malformation, and mental retardation
Rationale:
🧬 WAGR syndrome is a genetic disorder caused by a deletion in the WT1 gene, leading to:
Wilms tumor
Aniridia (absence of the iris)
Genitourinary anomalies
Intellectual disability (mental retardation)
🚫 Other options (B, C, D) do not correctly list the classic WAGR syndrome components.
98. Which of the following is the most common brain tumor in children?
A. Cerebellar astrocytoma
B. Ependymoma
C. Glioblastoma multiforme
D. Craniopharyngioma
A. Cerebellar astrocytoma
Rationale:
🧠 Cerebellar astrocytoma (pilocytic astrocytoma) is the most common pediatric brain tumor, typically presenting with ataxia and increased intracranial pressure.
🚫 Ependymoma (B) occurs in the ventricles.
🚫 Glioblastoma multiforme (C) is rare in children.
🚫 Craniopharyngioma (D) is more common in the suprasellar region.
99. A 15-year-old boy presents with a 3-month history of cervical swelling, fever, weight loss, and night sweats. The most likely diagnosis is:
A. Wilms’ tumor
B. Hepatoblastoma
C. Hodgkin lymphoma
D. Rhabdomyosarcoma
C. Hodgkin lymphoma
Rationale:
🩸 Hodgkin lymphoma classically presents with:
Painless cervical lymphadenopathy
B symptoms: fever, weight loss, night sweats
🚫 Wilms’ tumor (A) and hepatoblastoma (B) typically present with abdominal masses.
🚫 Rhabdomyosarcoma (D) usually presents as a soft tissue mass without systemic symptoms.
100. A 2-year-old girl presents with an abdominal mass, periorbital ecchymoses, and ataxia. The most likely diagnosis is:
A. Wilms’ tumor
B. Neuroblastoma
C. Brain tumor
D. Rhabdomyosarcoma
B. Neuroblastoma
Rationale:
🦠 Neuroblastoma presents with:
Abdominal mass (adrenal involvement)
Periorbital ecchymoses (“raccoon eyes”) due to metastatic disease
Ataxia (paraneoplastic effect: opsoclonus-myoclonus syndrome)
🚫 Wilms’ tumor (A) lacks periorbital bruising and ataxia.
🚫 Brain tumors (C) do not cause abdominal masses.
🚫 Rhabdomyosarcoma (D) typically presents as a localized soft tissue tumor.
1. A 15-year-old boy presents with a 3-month history of cervical swelling, fever, weight loss, and night sweats. The most likely diagnosis is:
A. Wilms’ Tumor
B. Hepatoblastoma
C. Hodgkin Lymphoma
D. Rhabdomyosarcoma
C. Hodgkin Lymphoma
Rationale:
🩸 Hodgkin lymphoma presents with painless cervical lymphadenopathy, accompanied by B symptoms (fever, weight loss, night sweats).
🚫 Wilms’ tumor (A) and hepatoblastoma (B) typically present with abdominal masses.
🚫 Rhabdomyosarcoma (D) usually presents as a soft tissue mass, not systemic symptoms.
2. The most common site for metastasis of osteosarcoma is:
A. Kidney
B. Lungs
C. Bone marrow
D. Liver
B. Lungs
Rationale:
🫁 The lungs are the most common site of metastasis for osteosarcoma due to hematogenous spread.
🚫 Bone marrow (C) and liver (D) are less commonly affected.
🚫 Kidneys (A) are not a primary site for osteosarcoma metastasis.
3. The most common solid tumors in children are:
A. Brain tumors
B. Renal tumors
C. Lymphomas
D. Sarcomas
A. Brain tumors
Rationale:
🧠 Brain tumors are the most common solid tumors in children, surpassing renal tumors and sarcomas.
🚫 Lymphomas (C) are hematologic, not solid tumors.
🚫 Sarcomas (D) and renal tumors (B) are less common than brain tumors.
4. Tumor lysis syndrome is most commonly associated with which of the following malignancies?
A. Neuroblastoma
B. Wilms’ Tumor
C. Hepatoblastoma
D. Leukemia and Lymphoma
D. Leukemia and Lymphoma
Rationale:
⚠️ Tumor lysis syndrome (TLS) occurs in malignancies with high cell turnover and rapid response to therapy, such as acute leukemias and high-grade lymphomas (e.g., Burkitt lymphoma).
🚫 Neuroblastoma (A), Wilms’ tumor (B), and hepatoblastoma (C) are not typically associated with TLS.
5. The best screening radiographic study for a patient with suspicious cervical lymphadenopathy, fever, and weight loss is:
A. Chest X-ray
B. Chest MRI
C. Abdominal CT scan
D. Neck X-ray
A. Chest X-ray
Rationale:
📸 A chest X-ray is the best initial imaging study for suspected lymphoma, detecting mediastinal masses.
🧲 MRI (B) and CT scan (C) are more detailed but not first-line screening tools.
🚫 Neck X-ray (D) is not useful for lymphadenopathy evaluation.
6. Which type of childhood leukemia carries the worst prognosis?
A. Adult-type CML
B. Juvenile CML
C. ALL L1
D. AML M1
B. Juvenile CML
Rationale:
🩸 Juvenile CML has a worse prognosis compared to adult-type CML and ALL L1.
🧬 ALL (C) has a better prognosis, especially in younger children.
🚫 AML M1 (D) has an intermediate prognosis but is not the worst.
7. Definitive diagnosis for sarcomas often requires:
A. Chest CT
B. Bone scan
C. Bone marrow biopsy
D. Tissue biopsy
D. Tissue biopsy
Rationale:
🔬 Definitive diagnosis of sarcomas requires a tissue biopsy to confirm malignancy and histologic subtype.
🚫 Chest CT (A) and bone scan (B) help with staging but do not confirm the diagnosis.
🚫 Bone marrow biopsy (C) is used in hematologic malignancies, not sarcomas.
8. Individuals with hereditary retinoblastoma have an increased risk for:
A. Neuroblastoma
B. Acute leukemia
C. Rhabdomyosarcoma
D. Osteosarcoma
D. Osteosarcoma
Rationale:
🧬 Hereditary retinoblastoma (RB1 gene mutation) is associated with an increased risk of osteosarcoma.
🚫 Neuroblastoma (A) and acute leukemia (B) are not strongly linked to hereditary retinoblastoma.
🚫 Rhabdomyosarcoma (C) is unrelated to RB1 mutations.
9. The most common clinical presentation of Hodgkin disease in children is painless lymph node enlargement in which region?
A. Mediastinal
B. Axillary
C. Cervical
D. Abdominal
E. Inguinal
C. Cervical
Rationale:
🩺 The most common clinical presentation of Hodgkin lymphoma in children is painless cervical lymphadenopathy.
🫁 Mediastinal involvement (A) occurs but is usually seen in older children and adolescents.
🚫 Axillary (B), abdominal (D), and inguinal (E) lymph nodes are less commonly affected initially.
10. Which of the following is the most common brain tumor in children?
A. Cerebellar astrocytoma
B. Ependymoma
C. Glioblastoma multiforme
D. Craniopharyngioma
A. Cerebellar astrocytoma
Rationale:
🧠 Cerebellar astrocytoma (pilocytic astrocytoma) is the most common pediatric brain tumor, typically presenting with ataxia and increased intracranial pressure.
🚫 Ependymoma (B) occurs in the ventricles.
🚫 Glioblastoma multiforme (C) is rare in children.`
🚫 Craniopharyngioma (D) is more common in the suprasellar region.
1. Which of the following viruses can cause aplastic crises in chronic hemolytic disease?
A. Herpes Virus
B. Human Parvovirus B19
C. Cytomegalovirus
D. T-lymphocyte virus
B. Human Parvovirus B19
Rationale:
🦠 Parvovirus B19 infects erythroid progenitor cells, causing transient red cell aplasia, leading to aplastic crises in chronic hemolytic diseases (e.g., sickle cell disease, hereditary spherocytosis).
🚫 Herpes virus (A), Cytomegalovirus (C), and T-lymphocyte virus (D) do not cause aplastic crises.
2. The role of corticosteroids in autoimmune hemolytic anemia is to decrease:
A. Immune response to offending organisms
B. Antibody production
C. The size of the spleen
D. The clearance of sensitized RBCs in the spleen
D. The clearance of sensitized RBCs in the spleen
Rationale:
🩺 Corticosteroids decrease macrophage-mediated clearance of antibody-coated RBCs in autoimmune hemolytic anemia.
🚫 They do not directly reduce antibody production (B) or spleen size (C).
🚫 They do not target infectious organisms (A).
3. Which hemoglobin is markedly elevated in beta thalassemia major?
A. Hb F
B. Hb A1
C. Hb A2
D. Hb H
A. Hb F
Rationale:
🩸 Beta thalassemia major is characterized by absent or severely reduced Hb A1 production, leading to compensatory elevation of fetal hemoglobin (Hb F).
🚫 Hb A2 (C) is mildly increased in β-thalassemia minor but not in major.
🚫 Hb H (D) is seen in α-thalassemia, not β-thalassemia.
4. Which of the following symptoms or signs is a manifestation of polycythemia?
A. Lymphadenopathy
B. Deep vein thrombosis
C. Epistaxis
D. Seizures
D. Seizures
5. Which of the following symptoms or signs is a manifestation of anemia?
A. Irritability
B. Fatigue
C. Bruising
D. Pharyngitis
B. Fatigue
Rationale:
🩸 Anemia leads to reduced oxygen delivery to tissues, causing fatigue as a common symptom.
😠 Irritability (A) can be seen in severe anemia but is not a primary symptom.
🔵 Bruising (C) is more indicative of platelet disorders or coagulopathies.
🦠 Pharyngitis (D) is related to infections, not anemia.
6. All of the following are treatment options for immune thrombocytopenia (ITP) EXCEPT:
A. Steroids
B. Androgens
C. Anti-D
D. Immunoglobulin
E. Splenectomy
B. Androgens
Rationale:
🩺 Immune thrombocytopenia (ITP) is managed with:
Steroids (A) to suppress the immune response.
Anti-D immunoglobulin (C) for Rh+ patients.
IVIG (D) to rapidly increase platelet count.
Splenectomy (E) in refractory cases.
🚫 Androgens (e.g., danazol) are not used in ITP but may be used in other hematologic disorders like aplastic anemia.
7. A feature that signifies β-thalassemia minor is:
A. Elevated hemoglobin A2
B. Absent marrow sideroblasts
C. High red cell distribution width
D. Low iron level
A. Elevated hemoglobin A2
Rationale:
🩸 β-thalassemia minor is characterized by a mild microcytic anemia with elevated Hb A2 (>3.5%) on hemoglobin electrophoresis.
🚫 Iron levels (D) are typically normal.
🚫 Marrow sideroblasts (B) are seen in sideroblastic anemia, not thalassemia.
🚫 Red cell distribution width (C) is usually normal in β-thalassemia minor.
8. The treatment of choice for most cases of type 1 von Willebrand disease is:
A. Desmopressin
B. vWF concentrate
C. Cryoprecipitate
D. Fresh frozen plasma
A. Desmopressin
Rationale:
💉 Desmopressin (DDAVP) is the treatment of choice for mild Type 1 von Willebrand disease (vWD), as it stimulates the release of stored vWF from endothelial cells.
🩸 vWF concentrate (B) is reserved for severe cases or surgery-related bleeding.
❌ Cryoprecipitate (C) and Fresh frozen plasma (D) are not preferred due to infection risk and inconsistent vWF levels.
9. A common significant feature of a blood smear in lead poisoning is:
A. Normochromic RBC
B. Macrocytic RBC
C. Target cells
D. Basophilic stippling
D. Basophilic stippling
Rationale:
🔬 Lead poisoning disrupts hemoglobin synthesis, causing ribosomal aggregation, visible as basophilic stippling on peripheral blood smear.
🚫 Normochromic (A) and macrocytic RBCs (B) are not characteristic.
🚫 Target cells (C) are seen in thalassemia, not lead poisoning.
10. In disseminated intravascular coagulation (DIC), which of the following is reduced?
A. Prothrombin time
B. Partial thromboplastin time
C. D-dimer
D. Bleeding time
E. Fibrinogen
E. Fibrinogen
Rationale:
⚠️ DIC is a consumptive coagulopathy leading to depletion of clotting factors, platelets, and fibrinogen.
📉 Fibrinogen levels are reduced due to excessive fibrin formation and breakdown.
📈 D-dimer (C) is increased due to fibrinolysis.
⏳ PT (A) and PTT (B) are prolonged due to clotting factor consumption.
🩸 Bleeding time (D) is increased but is not a specific marker for DIC.
