Images in Clinical Medicine: Cytoplasmic Blebs in T-Cell Prolymphocytic Leukemia

Summary

  • 64yo M
  • c/c
    • presented to emergency dep
    • 6mo Hx of
      • fatigue
      • dizziness
  • PEx
    • enlarged liver & spleen w/o lymphadenopathy
  • Ex
    • L/D
      • WBC 1279000/m3 (4000-11000)
        • Lym 100%
      • Hb 8.6g/dL (14-18)
      • Plt 24000/m3
    • PBS
      • intermediate-sized mature lymphoid cell
        • small nucleoi & lnobbing cytoplasmic blebs
    • flow cytometry
      • CD4+ CD8+
    • immunochemical analysis
      • TCL1
    • cytogenetic analysis
      • inv(14)(q11.2q32)
  • Dx
  • Tx

Further

Terminology

Original

Figure

A 64-year-old man presented to the emergency department with a 6-month history of fatigue and dizziness. A physical examination was notable for an enlarged liver and spleen without lymphadenopathy. Laboratory studies showed a white-cell count of 1,279,000 per cubic millimeter (reference range, 4000 to 11,000) with 100% lymphocytosis, a hemoglobin level of 8.6 g per deciliter (reference range, 14 to 18), and a platelet count of 24,000 per cubic millimeter (reference range, 140,000 to 440,000). A peripheral-blood smear revealed numerous atypical intermediate-sized mature lymphoid cells with small nucleoli and knobbing cytoplasmic blebs (Panel A). Flow cytometry showed a population of mature T-cells with CD4+ and CD8+ coexpression (Panel B). The neoplastic lymphocytes were positive for TCL1 as assessed by immunohistochemical analysis, and cytogenetic analysis revealed a complex karyotype that included inversion of chromosome 14(q11.2q32), with fluorescent in situ hybridization showing TCL1 rearrangement. A diagnosis of T-cell prolymphocytic leukemia was made, and the patient was admitted to the intensive care unit. He began treatment with glucocorticoids, and leukapheresis was initiated. Subsequently, he received alemtuzumab and pentostatin; by the time of a follow-up visit 3 months later, he was in complete remission, with residual malignant cells below the threshold of detection (negative minimal residual disease).