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.
Highlights
Hairy cell leukaemia (HCL) is a chronic B-cell leukaemia characterized by expansion of neoplastic cells in the spleen, bone marrow and blood
Hairy cell leukaemia (HCL) is a chronic B-cell leukaemia characterised by pancytopenia and clonal expansion of mature malignant B cells in the spleen, bone marrow and blood [1]
Patients with HCL do not suffer from leukaemia itself but from symptoms that are related to pancytopenia and opportunistic infections [2]
Summary
Hairy cell leukaemia (HCL) is a chronic B-cell leukaemia characterized by expansion of neoplastic cells in the spleen, bone marrow and blood. Case: We present a case of a 53-year-old female patient suffering from hepatosplenomegaly, peripheral lymphadenopathy and related B symptoms. Hairy cell leukaemia (HCL) is a chronic B-cell leukaemia characterised by pancytopenia and clonal expansion of mature malignant B cells in the spleen, bone marrow and blood [1]. Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of extranodal T-cell lymphoma derived from year-old female patient with coexistent HCL and HSTCL who was recently treated at our institution. Case report A 53-year-old female was referred to our centre because of a two-month lasting exhaustion, abdominal distension, early satiety and fever of up to 39°C. Laboratory tests demonstrated: (1) white blood count 3.40 × 109/l (Neu 2,01 (59%) , Ly 0,99 (29%), Mono 0,17 (5%), Eos 0,03 (1%)), platelet count 64 × 109/ l, red blood cells 3,66 × 109/l, haemoglobin level 115 g/l (2) liver function tests were raised: AST 2.21 IU/L, ALT 0.95 IU/L and γGT 1.30 IU/L with bilirubin within normal ranges-bilirubin 17 IU/L (3) LDH was raised to 15,68 IU/L; raised was CRP to 35 (4) serology for HAV showed reactive IgG anti-HAV antibodies, whereas IgM anti-HAV, HbsAg, anti- HBc, anti-Hbs, anti-HCV and CMV were negative
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