Coexistent hairy cell leukaemia and hepatosplenic t-cell lymphoma: a case report

Abstract

BackgroundHairy cell leukaemia (HCL) is a chronic B-cell leukaemia characterized by expansion of neoplastic cells in the spleen, bone marrow and blood. Symptoms of HCL are related to pancytopenia and immune deficiency. Patients with HCL have an increased risk of second malignancy either in a form of synchronous disease or in a form of an increased incidence of a second neoplasm after the treatment of HCL. Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of aggressive extranodal T-cell lymphoma. Its pathogenesis is connected to a chronic immune deficiency status and its coexistence with other neoplasms is practically non-existent.CaseWe present a case of a 53-year-old female patient suffering from hepatosplenomegaly, peripheral lymphadenopathy and related B symptoms. An excisional biopsy of the enlarged axillary lymph node revealed partial infiltration with CD3+/CD56+/TIA + T cell lymphoma. Bone marrow trephine biopsy and flow cytometric immunophenotypization of bone marrow cells and peripheral blood showed presence of two types of neoplastic cells in the peripheral blood and in the bone marrow (composite lymphoma). One of them showed typical morphologic characteristics and immunohistochemical features of HCL, while another one was morphologically and immunophenotypically consistent with the diagnosis of HSTCL, respectively. The patient was treated with multivalent chemotherapy including rituximab but all treatments turned out to be only partially effective. While HCL responded to the treatment, HSTCL was refractory to the chemotherapy and the patient died 7 months after the initial diagnosis because of haematemesis induced by Mallory-Weiss syndrome.ConclusionThis is the first recorded case of coexistent HCL and HSTCL in the same patient. A multidisciplinary approach, encompassing careful morphology interpretation, immunophenotypic, cytogenetic and molecular analyses, is mandatory to obtain an accurate diagnosis of composite lymphoma.Virtual slidesThe virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/9354870531161685.

<p><em>BRAF</em> V600E-positive monomorphic epitheliotropic intestinal T-cell lymphoma complicating the course of hairy cell leukemia</p>

“…The simultaneous occurrence of T- and B-cell neoplasms is exceptional and the etiologic and pathogenetic relationships between the two phenotypically different malignancies are not clear. An association between HCL and a T-cell NHL has been described in a few cases,9–11 but all the reported experiences could not provide any proof of a shared pathogenesis of the two conditions. MEITL, previously sub-classified as enteropathy-associated T-cell lymphoma type II, is a rare (<1% of NHL) and primarily gastrointestinal type of peripheral T-cell lymphoma with an aggressive clinical course and a poor prognosis.…”

Unusual Hairy Projections in a Case of T-acute Lymphoblastic Leukemia, a Cause for Diagnostic Dilemma: A Case Report

“…Our case also belongs to this category of non HCL cases showing the presence of circumferential hairy cytoplasmic projections resembling HCL and posing a diagnostic challenge. The issue was further complicated by several case reports in literature highlighting the coexistence of HCL in various haematological malignancies such as hepatosplenic T-cell non-Hodgkin lymphoma, Hodgkin lymphoma, peripheral T-cell lymphoma, multiple myeloma, chronic myeloid leukemia and chronic lymphocytic leukemia [4][5][6][7][8][9]. However in our case, immunophenotyping revealed most of the cells to be homogenously expressing the immaturity and T-cell phenotype marker confirming the diagnosis of T-ALL and ruling out the coexistence of HCL which otherwise could have contributed for this perplexing morphology.…”

Issues in diagnosis of small B cell lymphoid neoplasms involving the bone marrow and peripheral blood. Report on the Bone Marrow Workshop of the XVIIth meeting of the European Association for Haematopathology and the Society for Hematopathology

“…In BMW case 128, HCL was an incidental finding in a staging bone marrow biopsy in a patient with HSTCL. Case 128 is described in detail elsewhere …”