Leukemic phase of ALK-negative anaplastic large cell lymphoma in a patient who is on androgenic steroids: A case report

Abstract

IntroductionALK-negative anaplastic large cell lymphoma (ALCL) is a peripheral T-cell lymphoma that usually involves lymph nodes or extranodal sites. Leukemic phase of ALK-negative ALCL is exceedingly rare and often carries a poor prognosis. Androgenic steroids have gained popularity among the young, and at higher doses, it can result in immune dysregulation and may be potentially carcinogenic. Case presentation: A 30-year-old gentleman of Malay ethnicity presented to the hematology department with night fevers, loss of weight and bony pain for the past 6 weeks. He is a gymnasium instructor with a history of chronic usage of intramuscular testosterone enanthate. Physical examination revealed ecchymosis over the left elbow and hepatomegaly. A complete blood count depicted anemia, thrombocytopenia and leucocytosis. An 18-Fluorodeoxyglucose positron emission tomography (18-FDG PET/CT) imaging showed a hypermetabolic anterior mediastinal mass of 6.8 × 7.0 × 6.5 cm with diffuse hypermetabolism in the liver, spleen and axial skeleton. The bone marrow trephine and mediastinal tissue histology were consistent with leukemic ALK-negative ALCL. He was treated with CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, prednisolone) induction chemotherapy in which he required intensive antibiotic and blood support. He progressed with worsening B symptoms and new diffuse lymphadenopathies suggesting rapid dissemination of the disease. He subsequently succumbed to multiorgan failure with disseminated intravascular coagulopathy at the intensive care unit. Conclusion: Leukemic phase ALK-negative ALCL often carries a complex karyotype and requires early intensive polychemotherapy. Use of anabolic steroids depletes the ability of defending lymphocytes to remove tumour producing cells.

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

“…Some of the lymphoma cells in the blood or bone marrow aspirate smears had kidney-shaped nuclear contours, similar to the “hallmark” cells, as can be seen in the tissue sections. Most leukemic cases reported in the literature also resembled the common pattern with the exception of one case, in which a small cell morphology was described in a 30-year-old man with leukemic ALK-negative ALCL and had a history of chronic usage of androgenic steroids [ 15 ]. Although a small cell morphology is seen in a small subset of ALK+ ALCL cases [ 28 ], this variant is very rare and highly challenging to recognize in ALK-negative ALCL.…”

“…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.…”

Anaplastic Large Cell Lymphoma: Molecular Pathogenesis and Treatment

“…The involvement of other organs is usually shown as a mass. The presentation of leukemia is very rare in ALK−ALCL, foreshadowing a poor outcome [ 108 , 109 , 110 , 111 ].…”

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 ].…”