The masquerading presentation of a systemic anaplastic large cell lymphoma, ALK positive: a case report and review of the literature

Abstract

We report an unusual case of systemic anaplastic large cell lymphoma (ALCL), ALK positive with leukemic involvement in a 57-year-old woman. The patient presented with a fulminant respiratory infection unresponsive to treatment requiring intensive care and ventilatory support. The CT scan demonstrated mediastinal and bilateral lymphadenopathy. On peripheral smear a few atypical lymphocytes were visualized. Based on the increasing number of atypical lymphocytes in the daily peripheral bloodsmears, the diagnosis ALCL was suggested. Definitive diagnosis was made on a bone marrow biopsy, with lymphocytes being immunoreactive for CD30, EMA, and ALK. Leukemic peripheral blood involvement in ALCL is an uncommon clinicopathologic entity with unfavorable prognosis. The case we present is perhaps unusual in that a complete respons was achieved, highlighting the importance of prompt diagnosis and judicious management.

Pathological cytomorphologic features and the percentage of ALK FISH-positive cells predict pulmonary adenocarcinoma prognosis: a prospective cohort study

“…First identified in anaplastic large cell lymphomas, ALK is a receptor protein tyrosine kinase that activates many downstream signaling pathways, resulting in increased cell proliferation and survival [ 29 ]. Now, in many different cancers, such as neuroblastoma [ 30 ], and non-small cell lung cancer [ 31 ], rearrangements of ALK are found. This makes ALK an attractive target for cancer therapy.…”

Leukaemic Presentation of Small-Cell Alk-Positive Anaplastic Large Cell Lymphoma in a Young Woman—Report of a Case with 9-Year Survival

“…Although ALK-positive ALCL cases have a relatively good outcome, the small-cell leukemic variant is -irrespective of ALK-translocation status- an aggressive disease. A total of 35 cases of leukaemic, small cell variant of ALK-positive ALCLs have been previously reported [ 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ] ( Table 1 ) with limited follow-up time (from 1 to 63 months) in the surviving patients. All evidence points to an aggressive disease with a median overall survival of 12 months and a high tendency for central nervous system recurrences.…”