Cutaneous lesions in hairy-cell leukemia. Case report and review of the literature

Abstract

A patient is described in whom cutaneous lesions were the initial manifestation of hairy-cell leukemia. Touch preparations made immediately on removal of a 3-mm punch biopsy specimen of the cutaneous lesions revealed acid-phosphatase positive, tartrate-resistant staining in the leukemic cells, and helped to establish the diagnosis. Specific eruptions occur in approximately 7% of patients with hairy-cell leukemia, appearing grossly as disseminated, erythematous maculopapules, with a perivascular mononuclear leukemic cell infiltrate seen microscopically. A review of the English literature indicates that cutaneous manifestations are not generally recognized as a diagnostic source in individuals with hairy-cell leukemia, and biopsy is seldom undertaken.

Specific skin lesions in hairy cell leukemia at presentation: case report and review of literature

“…The patient reported by Bilsland et al [4] had elevated WBC the majority of which appeared to be hairy cells. Based on these data and clinical course of our patient we can no more state that cutaneous involvement regardles of stage of disease is not clearly poor prognosis indicator [2,3,13]. Leukemia cutis in all presented cases responded to the specific treatment of HCL with disappearing of cutaneous infiltrates.…”

“…Although skin leukemides in HCL occur during the course of the disease in about 8% of cases they are rare at presentation [2][3][4][9][10][11][12]. They have been described as evident at presentation only three times prior to the present report [2][3][4]. Carsuzaa et al [12] reported that 47 (56%) out of 84 HCL patients had cutaneous symptoms.…”

“…Bilsland et al [4] reported a patient with transient skin lesions at presentation who later developed pulmonary tuberculosis. In three reported cases in the literature [2][3][4] with specific skin lesions at presentation the number of WBC was low, normal and elevated, respectively. These three presented patients were treated with splenectomy and interferon-a and followed up from 12 to 45 months.…”

Diagnostic utility of SOX11 immunohistochemistry in differentiating cutaneous spread of mantle cell lymphoma from primary cutaneous B‐cell lymphomas

“…The blastoid variant of MCL has been suggested by multiple case reports to be associated with cutaneous involvement, has a worse clinical prognosis and is typically positive for SOX11 . A recent study examined a series of secondary cutaneous MCL and found that 12 of 14 (86%) cases were of the blastoid variant, with diffuse SOX11 staining seen in all of the cases in the series .…”

“…These lymphomas are either high grade B‐cell or T‐cell neoplasms and are morphologically and phenotypically distinct from MCL . Furthermore, SOX11 can also be positive in approximately 50% of hairy cell leukemia (HCL), which is a low‐grade B‐cell lymphoma that typically shows modest cyclin D1 staining and may be difficult to distinguish from MCL based on immunophenotype alone . However, SOX11 expression in HCL is very weak, in contrast to the strong staining seen in MCL .…”