Leukaemic relapse of anaplastic large cell lymphoma, ALK negative

Abstract

Anaplastic large cell lymphoma (ALCL), ALK negative (ALK−) is an aggressive lymphoproliferative disorder of mature T lymphocytes characterised by hallmark cells, CD30 positivity and lacking ALK protein expression. ALCL, ALK− has to be differentiated from peripheral T-cell lymphoma-not otherwise specified and classical Hodgkin’s lymphoma. ALK− anaplastic large cell leukaemia should be considered in a patient with a history of ALCL, ALK− presenting with new leukaemia. We report a rare presentation of relapsed ALCL, ALK− with leukaemia after autologous stem cell transplantation in a 57-year-old male. Leukaemia, either as primary presentation or secondary transformation confers worse prognosis in ALCL, ALK− with very few cases reported so far. Emergency resuscitation with leukapheresis and treatment of tumour lysis syndrome along with supportive care should be followed by combination chemotherapy. Brentuximab vedotin and stem cell transplantation are the backbone of treatment for relapsed/refractory disease.

The Leukemic Phase of ALK-Negative Anaplastic Large Cell Lymphoma Is Associated with CD7 Positivity, Complex Karyotype, TP53 Deletion, and a Poor Prognosis

“…The clinical features of the patients with leukemic ALK-negative ALCL in this study were similar to the patients with non-leukemic disease, except that the patients with leukemic disease had a higher frequency of bone marrow involvement, absolute lymphocytosis, and thrombocytopenia. In the literature, ten cases of leukemic ALK-negative ALCL have been reported prior to the current study: eight (80%) patients had leukemic disease at initial diagnosis and two patients developed a leukemic phase five years and twenty years after initial diagnosis, respectively [ 16 , 19 ]. Most of the reported patients also had bone marrow involvement and/or thrombocytopenia [ 16 , 17 , 18 , 19 , 20 , 21 ].…”

“…In the literature, ten cases of leukemic ALK-negative ALCL have been reported prior to the current study: eight (80%) patients had leukemic disease at initial diagnosis and two patients developed a leukemic phase five years and twenty years after initial diagnosis, respectively [ 16 , 19 ]. Most of the reported patients also had bone marrow involvement and/or thrombocytopenia [ 16 , 17 , 18 , 19 , 20 , 21 ].…”

“…Among the reported leukemic ALK-negative ALCL cases with follow-up data available, six of eight (75%) patients died, most within six months [ 15 , 16 , 17 , 18 , 20 , 34 ]. In the present study, the overall survival (OS) of the patients with leukemic ALK-negative ALCL was significantly shorter than that of the non-leukemic patients, confirming the poorer outcome of patients with leukemic phase ALK-negative ALCL.…”

“…The reported leukemic phase ALCL cases are almost all pediatric patients with ALK+ ALCL, which are most often associated with the morphologic features of small cell variant, t(2;5)(p23;q35), and a poorer prognosis [ 9 , 10 , 11 , 12 , 13 , 14 ]. Leukemic ALK-negative ALCL is extremely rare, with approximately ten cases reported in the literature to date, mostly as single case reports, and the patients had a poor outcome [ 15 , 16 , 17 , 18 , 19 , 20 , 21 ]. The clinicopathological features and molecular/genetic abnormalities underlying the leukemic phase of ALK-negative ALCL are largely unknown.…”

ALK-Negative Anaplastic Large Cell Lymphoma: Current Concepts and Molecular Pathogenesis of a Heterogeneous Group of Large T-Cell Lymphomas

“…The involvement of other organs usually occurs in the form of a mass. Leukemic presentation is very rare in ALK- ALCL and it portends a bad prognosis [ 24 , 25 , 26 , 27 ].…”