PA19.4 | Spleen Disorders — Graded Quiz

A 45-year-old man from rural Bihar presents with progressive abdominal distension for 6 months. Examination reveals massive splenomegaly (spleen tip 16 cm below costal margin), hepatomegaly, and mild ascites. Haemoglobin is 9.2 g/dL, WBC 3,400/µL, platelets 78,000/µL. Bone marrow aspirate shows normocellular marrow with adequate megakaryocytes. Serology for anti-rK39 is negative. Liver biopsy shows bridging fibrosis with regenerative nodules. Which single mechanism best explains the splenomegaly in this patient?

A 14-year-old girl with known β-thalassaemia major presents with worsening anaemia and progressively increasing transfusion requirements over 12 months (from 3-weekly to 2-weekly). Her spleen is massively enlarged (18 cm palpable). Haemoglobin between transfusions is 5.8 g/dL. Pre-transfusion haemoglobin used to be 8 g/dL. Alloantibody screen is negative. Which pathophysiological mechanism best explains her rising transfusion requirement?

A 55-year-old man presents with fatigue and early satiety. Spleen is palpable 20 cm below the costal margin. CBC shows WBC 85,000/µL with a differential showing the full spectrum of myeloid maturation (blasts 3%, promyelocytes 5%, myelocytes 18%, band forms and neutrophils 60%), eosinophilia, and basophilia. Platelet count is 620,000/µL. Haemoglobin is 10.1 g/dL. BCR-ABL fusion transcript is detected by RT-PCR. Which mechanism explains the splenic enlargement in this patient?

A 40-year-old man with rheumatoid arthritis for 15 years develops splenomegaly on routine examination. His CBC shows neutrophil count 900/µL, haemoglobin 9.8 g/dL, platelets 130,000/µL. He has no fever or lymphadenopathy. Antinuclear antibody is negative; rheumatoid factor is strongly positive. Bone marrow biopsy shows adequate myeloid precursors with normal maturation. Which diagnosis best explains this combination?

A 62-year-old man undergoes splenectomy for hereditary spherocytosis-related haemolytic anaemia. His preoperative blood film shows spherocytes. On day 2 post-splenectomy, the blood film shows the following new findings: small, dense nuclear remnants within red cells, target cells, and acanthocytes. Platelet count has risen to 680,000/µL. Which of the following post-splenectomy risks requires the most urgent preventive intervention?

A 16-year-old boy is brought to the emergency department during the monsoon season with high-grade fever, severe headache, and altered consciousness. Blood film shows multiple ring-trophozoites within a single red cell and banana-shaped gametocytes. His spleen is massively enlarged (16 cm palpable). Haemoglobin is 7.2 g/dL. Which pathological process in the spleen is responsible for sequestration of parasitised red cells and contributes most directly to organ damage in this infection?

A 52-year-old man presents with fatigue and abdominal discomfort. Examination reveals massive splenomegaly (21 cm palpable below costal margin), pallor, and no lymphadenopathy. Bone marrow aspiration yields a 'dry tap'; trephine biopsy shows hypercellular marrow with markedly increased reticulin fibrosis (grade 3/3) and bizarre megakaryocytes in clusters. WBC is 19,000/µL with a left shift and teardrop red cells (dacryocytes) on film. Serum LDH is elevated. Which mechanism most directly explains the massive splenomegaly in this patient?

A 50-year-old woman is evaluated for splenomegaly detected incidentally. She has dry eyes, dry mouth, and bilateral parotid swelling. Serum anti-Ro and anti-La antibodies are positive. CBC shows WBC 2,800/µL (absolute neutrophil count 1,100/µL), haemoglobin 10.9 g/dL (normocytic), and platelets 98,000/µL. Bone marrow biopsy shows normal cellularity. Which mechanism best explains her splenomegaly?

A 38-year-old man from Kerala presents with splenomegaly (12 cm palpable), hepatomegaly, and generalised lymphadenopathy. He has a history of recurrent skin rashes and is known to be HTLV-1 seropositive. His peripheral blood film shows hypersegmented lymphocytes with convoluted ('flower') nuclei. Which category of splenomegaly best describes his condition and what is the mechanism?

A 10-year-old girl presents with progressive splenomegaly since infancy, bone pain, and easy fatigability. Examination reveals massive splenomegaly (18 cm palpable), hepatomegaly, and mild coarse facies. Bone marrow biopsy shows large macrophages with pale, crinkled ('tissue paper') cytoplasm. Enzyme assay confirms glucocerebrosidase deficiency. Her blood film shows pancytopenia. Which statement best explains the mechanism of her massive splenomegaly?

A 25-year-old man who underwent splenectomy 2 years ago for trauma now presents to the emergency department with sudden onset high-grade fever (40°C), rigors, confusion, and hypotension developing over 4 hours. He received no post-splenectomy vaccinations. His peripheral film shows no Howell-Jolly bodies being phagocytosed (as expected). Blood cultures are taken. Which organism is MOST likely responsible for this presentation, and why is this patient at particular risk?

A 40-year-old woman with a 4-year history of systemic lupus erythematosus (SLE) on hydroxychloroquine develops splenomegaly (8 cm below costal margin) and increasing fatigue. CBC shows haemoglobin 8.4 g/dL (normocytic), WBC 2,200/µL, platelets 62,000/µL. Bone marrow biopsy shows adequate cellularity with normal maturation. The ALL criteria for hypersplenism are met. Which treatment approach would directly address the mechanism of her cytopenias?