Hairy Cell Lymphoma: A Potentially Under-Recognized Entity

Abstract

Hairy cell leukemia (HCL) is a low grade B-cell lymphoproliferative disorder that typically presents with splenomegaly, cytopenias, and diffuse bone marrow infiltration. There have been few cases in the literature of HCL presenting as lymphomas in extra-nodal locations, such as soft tissues and bones without circulating leukemic cells, splenomegaly, or iliac crest bone marrow involvement. We present an additional case presenting as a thoracic mass, and discuss potential diagnostic pitfalls and management of these rare cases.

Bone lesions in hairy cell leukemia: Diagnosis and treatment

“…However, recent reports from the era of purine analog therapy in HCL indicate that there is no difference no difference in the outcome of patients with and without bone lesions 11 . Rituximab appears to be a promising agent for relapsed patients and can be used as monotherapy or in combination with PNA 43,44 . The immunotoxin moxetumomab pasudotox and the BRAF inhibitors vemurafenib and dabrafenib have also been shown to be effective, especially in patients with limited responses to conventional therapies or treatment failure 39‐41 .…”

“…In an early report, persistent pain resolution was achieved in two patients treated with RT: 60 Gy in 30 fractions or 8 Gy in a single fraction 14 . Elsewhere, pain relief was achieved by RT administered to pathologic sites to a total dose of 15 ‐ 60 Gy, 15,21,30,43 and at doses varying from 15 to 30 Gy 10 . Local RT with an “adapted” dose and fractionation schedule in the range of 20‐30 Gy has been suggested for long‐lasting symptom control 11 .…”

“…11 Rituximab appears to be a promising agent for relapsed patients and can be used as monotherapy or in combination with PNA. 43,44 The immunotoxin moxetumomab pasudotox and the BRAF inhibitors vemurafenib and dabrafenib have also been shown to be effective, especially in patients with limited responses to conventional therapies or treatment failure. [39][40][41] These agents can further improve the prognosis of skeletal HCL, but there are currently no reports on their use in such cases.…”

Hairy Cell Leukemia (HCL) Presenting with Joint Swelling: Case Report and Literature Review of a Rare Rheumatological Manifestation of a Hematological Disease

“…Rarely has HCL also presented in the form of lymphomas in bones and soft tissues [8]. A retrospective review of 37 cases by Westbrook and Golde reported that six patients had joint symptoms [9].…”

“…This presentation has been attributed to either the hematological malignancy itself or an immune dysfunction [10]. This array of varying manifestations has often led to diagnostic uncertainty and potential underdiagnosis of this malignancy [8,10]. Furthermore, various case reports have described the presentation of RA with HCL.…”