Images in Clinical Medicine: Massive Splenomegaly

Summary

  • 35yo M
  • c/c
    • 3w Hx of
      • fatigue
      • painful distention of Lt side of abd
  • PMH
    • hepatosplenic T-cell lymphoma
  • PEx
    • massive enlargement of liver & spleen
  • Ex
    • CT
      • hepatomegaly
      • splenomegaly
    • L/D
      • ↑WBC 17400/mm3 (4000-11000)
      • ↓Hb 3.9g/dL (13.3-17.7)
      • ↓Plt 10000/mm3 (150000-450000)
    • smear
      • atypical mononuclear
        • intermediate to large in size
        • irreg nuc contours
        • moderatorely fine chromatin
        • prominent nucleoli
        • cytoplasmic blebs
    • flowcytometry
      • relapse hepatosplenic T-cell lymphoma
  • DDx
    • massive splenomegaly
      • chronic myeloid leukemia
      • myelofibrosis
      • hairy-cell leukemia
      • malaria, β-thalassemia major
      • visceral leishmaniasis
  • Tx
    • immune checkpoint blockade
    • opioid analegesics
  • Progress
    • presentation +10w died

Further

Terminology

Original

Figure

A 35-year-old man presented to the emergency department with a 3-week history of fatigue and painful distention of the left side of his abdomen. He had a history of hepatosplenic T-cell lymphoma and had completed treatment 9 months earlier. Physical examination revealed massive enlargement of the liver and spleen, with the spleen crossing the midline and its lower margin extending into the pelvis. A computed tomographic scan of the abdomen confirmed hepatomegaly and splenomegaly (with the spleen measuring 36 cm in its greatest dimension). The white-cell count was 17,400 per cubic millimeter (reference range, 4000 to 11,000), the hemoglobin level 3.9 g per deciliter (reference range, 13.3 to 17.7), and the platelet count 10,000 per cubic millimeter (reference range, 150,000 to 450,000). A blood smear showed atypical mononuclear cells, intermediate to large in size, with irregular nuclear contours, moderately fine chromatin, prominent nucleoli, and some cytoplasmic blebs. Relapsed hepatosplenic T-cell lymphoma was confirmed on flow cytometry. Other causes of massive splenomegaly include chronic myeloid leukemia, myelofibrosis, hairy-cell leukemia, malaria, β-thalassemia major, and visceral leishmaniasis. Treatment with immune checkpoint blockade and opioid analgesics was initiated, but the patient died consequent to disease progression 10 weeks after presentation.