Anaplastic large cell lymphoma in leukemic phase: extraordinarily high white blood cell count.
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.
Nguyen
Jacqueline T
JT
Department of Pathology and Laboratory Medicine, University of Texas-Houston Medical School, Houston, Texas, USA.
Condron
Michael R
MR
Nguyen
Nghia D
ND
De
Jitakshi
J
Medeiros
L Jeffrey
LJ
Padula
Anthony
A
D000328:Adult; D000077548:Anaplastic Lymphoma Kinase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002869:Chromosome Aberrations; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007958:Leukocyte Count; D017728:Lymphoma, Large-Cell, Anaplastic / Q000097:blood* / Q000235:genetics / Q000473:pathology*; D008297:Male; D016133:Polymerase Chain Reaction; D011505:Protein-Tyrosine Kinases / Q000378:metabolism; D020794:Receptor Protein-Tyrosine Kinases
C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases