Clinico-pathological profile of Hairy cell leukemia: Critical insights gained at a tertiary care cancer hospital.

Context: Hairy cell leukemia (HCL) is a rare, low grade, B-cell neoplasm with a characteristic morphologic and immunophenotypic profile. It has to be distinguished from chronic lymphoproliferative disorders because of different treatment protocol and clinical course. Aims: To evaluate clinicopathological features including immunophenotypic analysis of cases diagnosed as HCL. Materials and Methods: The present study included 28 cases diagnosed over a period of nine years (2002-2010). Clinical presentation, complete blood count, bone marrow aspirate, and flow cytometric analysis of cases were reviewed. Treatment and follow-up details (ranging from 3-90 months) were noted. Results: This study revealed 28 cases (referrals-7, indoor-21), aged 26-69 years with a median age of 47 years, with a male predominance (M:F=6:1). The presenting complaints were weakness (80%) followed by fever (56%) and abdominal pain. Physical examination revealed splenomegaly in most patients (92%) and hepatomegaly in a minority (28%). The common laboratory features were anemia in 23 cases, pancytopenia in 14 cases, while two patients had leukocytosis and three patients had normal WBC count. Dry tap was observed in 84% of the cases where hairy cells constituted 16-97% of non-erythroid nucleated cells. Tartarte resistant acid phosphate staining was positive in all the eight cases where it was done. CD5 was negative in all the cases, while CD10 was expressed in three cases (13%) and CD23 in five cases (19%). Conclusions: Though pancytopenia is common, occasional patient can present with normal blood counts or leukocytosis. Few unusual findings include presence of lymphadenopathy, absence of palpable splenomegaly, and expression of CD23 and CD10 by the leukemic cells.

Clinico-pathological presentation and therapeutic options for hairy cell leukemia

To look at clinical and hematological presentation as well as treatment outcome of patients with diagnosis of Hairy Cell Leukemia [HCL] in our population. All patients diagnosed with HCL by morphological and immunohistochemical methods presenting to Medical Oncology Ward at Hayatabad Medical Complex, Peshawar since August 2008 were included in the study. Out of 7 patients diagnosed with HCL, 6 were male and 1 was female [M:F ratio 6:1]. Median age at diagnosis was 44 years. Fever, pallor, palpitations and fatigue were the commonest presenting complaints. Spleenomegaly was noted in all patients [100%]. Pancytopenia was noted in all patients at presentation. Five patients were treated with Interferon while 2 were treated with Cladribine. At 18 months of follow-up, one out five patients treated with IFN had relapse while both the patients treated with Cladribine were in complete remission. Our study showed that HCL patients present at a younger age in our region but further studies with larger sample size are required to confirm this. All patients showed a complete response to Interferon and Cladribine with all patients alive at 18 months of follow-up.

Leucemia de células velludas en el embarazo: Caso clínico / Hairy cell leukemia during pregnancy: Report of one case

Hairy cell leukemia (HCL) is a rare chronic B cell lymphoproliferative disorder that affects mostly men. It usually presents with pancytopenia, splenomegaly and bone marrow infiltration, without lymphadenopathy. Diagnosis is based on the presence of mononuclear cells with cytoplasmic projections in a blood smear, the typical bone marrow infiltration pattern and the immunophenotypic profile. HCL occurs seldom in young women and even more exceptionally during pregnancy. We report a 31-year-old woman in whom a splenomegaly was detected during routine prenatal care. Pancytopenia with 25 percent of hairy cells was found in her blood count. The patient was subjected to an open splenectomy and had an uneventful pregnancy. After two years of follow up, she has a normal blood count and has not required chemotherapy.

Rituximab, 2a línea de tratamiento en leucemia de células vellosas / Rituximab 2nd line of treatment for hairy cell leukemia

La leucemia de células vellosas (LCV) es un desorden linfoproliferativo crónico caracterizado por: esplenomegalia, pancitopenia con linfocitos vellosos e infiltración de médula ósea y de bazo. Rituximab demuestra eficacia terapéutica y en pacientes con LCV refractaria/recaída.

Hairy cell leukaemia in Oman four cases

Hairy cell leukaemia [HCL] is a rare, clonal, chronic lymphoproliferative disorder commonly seen in males in the middle years of life. Pancytopaenia with moderate to massive splenomegaly is the most common clinical presentation. Diagnosis is made on detecting the lymphocytes with abundant cytoplasm which spread into hair-like processes on peripheral blood and bone marrow smears, thus giving the name, 'hairy cell leukaemia'. The bone marrow aspirate is frequently a dry tap. The trephine biopsy has the characteristic features of a honey comb appearance and flow cytometry is typically CD[103], CD[25], FMC[7], CD[11c], gamma or kappa light chain positive with the classic B lymphocyte markers CD[19], CD[20], CD7[9a]. Purine analogues followed by granulocyte-colony stimulating factor [G-CSF] to manage the febrile neutropenia is currently the treatment of choice. A 10 year disease free survival is recorded with these management strategies. Experimental use of anti CD[20] and CD[22] has also shown promising results in the treatment of this disease. We report four cases of HCL diagnosed in a span of two years at the Royal Hospital, Muscat, Oman

Hairy cell leukemia: a histo-cytochemical and ultra-structural study

We studied five patients with hairy cell leukemia (HCL) diagnosed within the last ten years at the Department of Hematology of Universidade Federal de Sao Paulo - Escola Paulista de Medicina. Our purpose was to analyze the value of transmission electron microscopy (TEM) by comparing this method with the conventional ones. At diagnosis, patients presented weight loss, spleen enlargement and hairy cells (HC) in peripheral blood and bone marrow slides. HC was characterized by morphology and tartrate test resistance in the acid phosphatase reaction (TRAP). At the evaluation time, the amount of HC ranged from 1 per cent to 85 per cent of WBC count. All patients, except two, had phenotype B. In these last two, TRAP as well as phenotype B could not be documented due to low HC numbers in their exams. Cytoplasmatic projections and the absecence of lamellar ribosomic complex were the most frequent ultrastructural findings, even in those patients with the lowest HC numbers. Based on these features, TEM is an efficient method for searching for HC at HCL diagnosis and during the course of the disease.

A clinico-pathological study of six cases of hairy cell leukaemia.

Clinicopathological findings of six cases of Hairy cell leukaemia are presented. All the patients were males, the age ranged between 32-57 years. Complications of anaemia and neutropenia were common modes of presentation. Hepatomegaly and splenomegaly were present in all the cases whereas only 2 patients had lymphadenopathy. Severe pancytopenia was detected in 3 cases and circulating hairy cells were present in all the cases. Trephine biopsy done in all six patients was found to be diagnostic. Tartrate resistant acid phosphatase was detected in the hairy cells of 2 cases.

Leucemia de células vellosas: complicación dermatológica / Hairy-cell leukaemia: dermatologic complication

Se comunica un paciente de 49 años, sexo masculino, con lesión erosiva impetiginizada en región pubiana y ulceración no infiltrada en prepucio. Masa ganglionar inguinal derecha flegmásica de 15 x 5 x 2,5 cm. Micropoliadenopatías inguinales izquierdas y adenopatía asintomática axilar derecha. Hepatoesplenomegalia. Astenia, Pancitopenia. Los estudios realizados ratificaron el diagnóstico de L. C. V., cuya frecuencia es del 2% en el total de las leucemias del adulto. La C. V. tiene su origen en una célula linfoide B y se sitúa en el sistema de diferenciación en un estadio algo más evolucionado que la de la leucemia linfática crónica. Se caracteriza por tener citoplasma extenso dotado de típicas prolongaciones o pelos. La maior parte del cuadro clínicos es consecuencia de la pancitopenia y la explenomegalia. las lesiones dermatológicas asociadas más frecuentemente son las inespecíficas, y se expresan por infecciones recurrentes, equimosis o las resultantes de vasculitis. Las específicas se observan en un 8% de los casos y se traducen por erupciones máculo-pápulo eritematosas. predomina en el sexo masculino, pero el pronóstico es mejor en el femenino. Los tratamientos instituídos segun la sintomatología y curso evolutivo son la esplenectomía, el alfa Interferón, y 2 - Deoxycoformicina

Value of splenic aspirate in the diagnosis of hairy cell leukaemia.

We present a case with hairy cell leukaemia which was diagnosed on a splenic aspirate. The patient refused splenectomy.

Hairy cell leukemia.

Six patients of 'Hairy cell leukemia' (HCL) were seen by us between 1982 and 1987. Five patients had anaemia and two had leukopaenia. None of the patients had thrombocytopaenia at presentation. All the patients were positive for tartarate resistant acid phosphatase. Two had B and one had T cell surface markers. Characteristic cytoplasmic projections and ribosomal lamellar complexes were seen on electron microscopy in four patients. Three patients were treated with splenectomy with satisfactory results.