Anaplastic large cell lymphoma in leukemic phase: Extraordinarily high white blood cell count

Abstract

Anaplastic large cell lymphoma (ALCL) is a distinct type of T/null-cell non-Hodgkin lymphoma that commonly involves nodal and extranodal sites. The World Health Organization of lymphoid neoplasms recognizes two types: anaplastic lymphoma kinase (ALK) positive or ALK negative, the former as a result of abnormalities involving the ALK gene at chromosome 2p23. Patients with ALCL rarely develop a leukemic phase of disease, either at the time of initial presentation or during the clinical course. Described herein is a patient with ALK+ ALCL, small cell variant, associated with the t(2;5)(p23;q35), who initially presented with leukemic involvement and an extraordinarily high leukocyte count of 529 x 10(9)/L, which subsequently peaked at 587 x 10(9)/L. Despite chemotherapy the patient died 2(1/2) months after diagnosis. In the literature review 20 well-documented cases are identified of ALCL in leukemic phase reported previously, with a WBC ranging from 15 to 151 x 10(9)/L. Leukemic phase of ALCL occurs almost exclusively in patients with ALK+ ALCL, most often associated with the small cell variant and the t(2;5)(p23;q35), similar to the present case. Patients with leukemic phase ALK+ ALCL appear to have a poorer prognosis than most patients with ALK+ ALCL.

Leukemic Presentation of ALK-negative Anaplastic Large Cell Lymphoma in a Patient with Myelodysplastic Syndrome

“…Its clinical features include significant respiratory distress, diffuse lung infiltrates or pleural effusions, and hepatosplenomegaly. Although ALK positivity is generally associated with a favorable outcome, leukemic ALK-positive ALCL has a highly aggressive clinical course and a poor outcome (16)(17)(18). We were able to closely follow the clinical course of the present patient as she regularly visited our hospital for the treatment of pancytopenia; we observed that the development and progression of the lymphoma was remarkably rapid in the leukemic phase.…”

Unusual Presentation of a Small-Cell Variant of Anaplastic Large-Cell Lymphoma Case: When a Septic Picture Is Not Sepsis

“…While leukemic peripheral blood involvement is rare in ALCL, an association has been reported with small-cell variants [5, 6, 11], which may be a potential explanation for the poor prognosis and aggressive nature of small-cell variant ALCL. Extreme leukocytosis involving neutrophils and atypical lymphocytes is usually the feature noted in the peripheral blood.…”

Intravascular T‐cell lymphoma: A rare, poorly characterized entity with cytotoxic phenotype

“…Unlike intravascular B‐cell lymphoma, a well‐defined entity, intravascular T‐cell lymphoma is not included in the current classification of lymphomas and its revision, probably due to the very small number of cases thoroughly characterized and reported. Moreover, most cases reported as intravascular T‐cell or natural killer (NK)‐cell lymphomas actually are leukemic presentation or variants of other types of lymphoma, mainly NK/T‐cell lymphoma, anaplastic large cell lymphoma (both anaplastic large cell kinase positive and negative types), or a recently described CD30 positive intravascular T‐lymphoproliferative disorder involving the skin . An additional level of complexity is added by the wide geographical distribution of the cases and by the lack of a homogenous work‐up.…”