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Atypical blood and bone marrow lymphocytes in ALK-positive anaplastic T-cell lymphoma: important lessons

A J Mahdi1, N Elmusharaf, H Osman

  • 1Department of Haematology, Royal Gwent Hospital, Newport, UK.

BMJ Case Reports
|September 17, 2013
PubMed

Insights

Anaplastic T-cell lymphoma, ALK-positive, presented atypically in peripheral blood and bone marrow without standard markers. Despite treatment, the patient relapsed and succumbed to pancytopenia within a year.

Area of Science:

  • Hematology
  • Oncology
  • Immunopathology

Background:

  • Anaplastic T-cell lymphoma (ATL) is a distinct non-Hodgkin lymphoma subtype.
  • Anaplastic lymphoma kinase (ALK) positivity is a key diagnostic and prognostic marker in ATL.
  • This case highlights an unusual presentation of ALK-positive ATL.

Observation:

  • A 42-year-old male presented with B symptoms (fever, weight loss) and lymphadenopathy.
  • CT revealed widespread lymphadenopathy and hepatosplenomegaly.
  • Peripheral blood and bone marrow showed atypical lymphocytes, but lacked typical ALK and CD30 markers.

Findings:

  • Diagnosis of ALK-positive anaplastic T-cell non-Hodgkin's lymphoma was confirmed via lymph node biopsy.
  • Initial chemotherapy induced a short remission.
  • Relapse occurred in peripheral blood and bone marrow despite salvage chemotherapy and autologous stem cell transplant, leading to pancytopenia and death.

Implications:

  • This case underscores the importance of comprehensive immunophenotyping and immunohistochemistry in diagnosing challenging lymphoma cases.
  • The absence of typical markers in PB and BM despite systemic disease suggests potential diagnostic complexities.
  • The aggressive clinical course and treatment resistance emphasize the need for further research into novel therapeutic strategies for refractory ALK-positive ATL.

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