Clinico-pathological and Immunophenotype Analysis of Hairy Cell Leukemia Cases – A Single Institute Experience

Background: Hairy cell leukemia is a mature lymphoid B cell disorder, characterized by hairy cells, a specific genetic profile, different clinical course and the need for an appropriate treatment. It is seen primarily in elderly, characterized by a triad of splenomegaly, pancytopenia and monocytopenia. Aim: To evaluate clinicopathologically and with immunophenotyping hairy cell leukemia cases received at our institute in conjunction with similar studies. Material and methods: This is a retrospective study which included 7 cases over a period of 3 years (2019-2021) confirmed on morphology and flow cytometry. Results: The study revealed 7 cases which showed patients with age ranging from 34-65 years. M:F ratio was 6:1. Two cases were covid positive (28.5%). Most of the cases presented with fever, weakness (28.5%). Splenomegaly was seen in three of the cases (42.6%). Laboratory investigations revealed anemia in 71% cases, leucopenia in 56.8%, lymphocytic prominence in 100% and pancytopenia in 14.2%. One patient presented with leukocystosis (14.2%). Marrow was hemodiluted and aparticulate in 3(42.6%) cases. Hairy cells were seen on morphology of peripheral smear and marrow aspirate. On flow cytometry, CD5 negative in all cases (100%), CD10 positive in 2(28.5%) and CD23 in 2 cases (28.5%). Few cases confirmed BRAF v600e mutations. Conclusion: Unusual findings like leukocytosis, absence of spleen, presence of lymphadenopathy can be present in hairy cell leukemia. Classical fried egg appearance in trephine biopsy may not be afeature in all the cases. CD123 is expressed in covid patients unlike other studies and further research is needed to establish the loss of CD123 in covid patients.

Clinicopathological spectrum of hairy-cell leukemia: A single-center study with brief review of Indian literature

Objective: The presence of specific chemotherapeutic protocols for hairy cell leukemia (HCL) makes it essential to discriminate this entity from other lymphoproliferative disorders. Hence, awareness of the variations in clinical presentations and immunophenotypic aberrancies is requisite to ensure diagnostic accuracy. Materials and Methods: A retrospective study was carried out to analyze the clinical-pathological profile of patients with HCL diagnosed over a period of 81 months (2010–September 2017) in our institute. Flow cytometry was performed in all the patients, and further, BRAFV600E mutation analysis was performed by real-time polymerase chain reaction in a limited number of samples. Result: A total of 353 lymphoproliferative disorders were assessed during the period, of which 16 (4.5%) were diagnosed as HCL, which included 15 cases of classical HCL and single case of HCL-v. Striking male predominance was noted with a median age of 52 (range 22–90 years). 47% patients presented with pancytopenia, while 20% cases had leukocytosis. Three patients presented with bleeding diathesis in the form of melena and purpuric spots. The absence of splenomegaly was observed in 20% patients (4/15) while 2 (13.3%) cases had lymphadenopathy. Hypocellular marrow was observed in 13% cases. Bright expression of CD20/CD22 along with CD25/CD103/CD123/CD11c was noted in all the patients of classical HCL. Aberrant expression of CD23 and CD5 was seen in 33% ( n =5) and 6.7% ( n =1) cases respectively. CD200 was positive in all the 5/15 cases tested. The case of HCL–v presented with very high leukocyte count and exhibited a CD103/CD11c+ and CD123/CD25- profile. BRAFV600E, mutation was present in all the four patients tested who included patients with a hypocellular marrow and absent splenomegaly. Conclusion: HCL has characteristic profiles, yet it may exhibit unusual clinical and immunophenotypic presentations. Perspicacious use of flow cytometry and BRAFV600E mutation analysis will aid in the diagnosis in unprecedented cases

Outcome of hairy cell leukemia patients treated with cladribine - a 10-year single-center experience in Pakistan

ABSTRACT Introduction and objective: Hairy cell leukemia is an uncommon, indolent B-cell lymphoproliferative disorder. Therapy with cladribine (2-chlorodeoxyadenosine) is able to induce complete remission (CR) in the majority of patients after a single course of treatment. We report the outcomes of patients treated at Aga Khan University Hospital, Karachi, Pakistan. Methods: This was a retrospective review. Medical records of patients were used to collect data. Results: A total of 21 patients with hairy cell leukemia were treated with cladribine. All patients achieved an initial CR. Four patients (19%) required hospitalization and therapy for neutropenic fever. Six patients (29%) relapsed at a median of 48 months. All 6 patients were treated for relapse, out of which 4 achieved CR, 1 had partial response and 1 had refractory disease. The overall survival rate was 90.5%, with a median follow-up of 35 months. Conclusion: A single course of cladribine is able to induce CR in a vast majority of patients. Unfortunately, relapse is not uncommon. Patients who relapse can be successfully retreated with cladribine. Cladribine has impressive efficacy and a favorable acute and long-term toxicity profile when administered to patients with HCL.

“Hairiness” is a Facsimile of Reorganized Cytoskeletons: A Cytopathic Effect of Coxiella burnetii

In 1993, I reported that Coxiella burnetii transforms human B cells into hairy cells (cbHCs), the first hairy cell reported outside of hairy cell leukemia (HCL). Over last few decades, advances in molecular biology have provided evidence supporting that C. burnetii induces hairiness and inhibits the apoptosis of host cells. The present review summarizes new information in support of cbHC. C. burnetii was shown to induce reorganization of the cytoskeleton and to inhibit apoptosis in host cells. Peritoneal B1a cells were found to be permissive for virulent C. burnetii Nine Mile phase I (NMI) strains in mice. C. burnetii severely impaired E-cad expression in circulating cells of Q fever patients. B-cell non-Hodgkin lymphoma was linked to C. burnetii. Mutation of BRAF V600E was pronounced in HCL, but “hairiness” was not linked to the mutation. Risk factors shared among coxiellosis and HCL in humans and animals were reported in patients with Q-fever. Accordingly, I propose that C. burnetii induces reorganization of the cytoskeleton and inhibits apoptosis as cytopathic effects that are not target cell specific. The observed hairiness in cbHC appears to be a fixed image of dynamic nature, and hairy cells in HCL are distinct among lymphoid cells in circulation. As the cytoskeleton plays key roles in maintaining cell structural integrity in health and disease, the pathophysiology of similar cytopathic effects should be addressed in other diseases, such as myopathies, B-cell dyscrasias, and autoimmune syndromes.

Efficacy of Annexin A1 Immunostaining in Bone Marrow for the Diagnosis of Hairy Cell Leukemia

Splenic B-cell lymphomas (SBCLs) show characteristically pronounced splenomegaly without significant lymphadenopathy. Distinguishing hairy cell leukemia (HCL) from other SBCLs (splenic marginal zone lymphoma [SMZL], variant HCL [v-HCL], and splenic diffuse red pulp small B-cell lymphoma [SDRPL]) is essential to determine suitable treatments and prognoses. With advances in diagnostic modalities and therapies, splenectomy is not commonly performed, and thus diagnosis of HCL must be based on the results obtained using blood and bone marrow samples. Annexin A1 is known as the most specific marker for HCL. There has yet been no report of the assessment of annexin A1 immunostaining from Korea. In this study we analyzed samples from 13 Korean patients with SBCLs (three HCL, three v-HCL, six SMZL, and one SDRPL) from May 2001 to December 2016. Immunohistochemical analyses for annexin A1 and CD20 were performed using bone marrow sections; molecular analyses for detection of the BRAF V600E mutation were also performed. All HCL patients showed positive results for annexin A1 immunostaining and the presence of the BRAF V600E mutation, and negative results for other SBCLs. Our results confirmed the high specificity of annexin A1 and the BRAF V600E mutation as HCL markers. Molecular analysis requires expensive equipment and substantial manpower. Annexin A1 is a better alternative as an HCL marker than the BRAF V600E mutation in terms of cost-effectiveness.

BRAFV600E mutation in hairy cell leukemia: A single-center experience

Background: BRAFV600E mutation has been reported as a unique genetic lesion of hairy cell leukemia (HCL), a subset of which lacks this lesion and shows adverse outcomes. Aims: To determine the prevalence of BRAFV600E in HCL from our center and derive clinicopathological correlation, if any. Materials and Methods: A 9-year retrospective analysis of 46 consecutive cases of HCL diagnosed on morphology and immunophenotyping was done. Stained smears were used as samples for amplification refractory mutation system polymerase-chain reaction using fluorescent primers for mutation detection. Results: BRAFV600E mutation was detected in 41/46 patients (89.1%) while absent in control samples of chronic lymphocytic leukemia. Cases mimicking HCL-variant clinically or immunophenotypically too showed the presence of this mutation. HCL with mutated BRAF presented at a younger age. No statistical difference in blood counts, tumor load, and immunophenotype patterns existed among BRAF mutated and unmutated group. Nine patients (45%) with mutated BRAF had residual disease following treatment with cladribine. Conclusion: BRAFV600E mutation analysis has a definitive role in the diagnosis of HCL.

Fludarabine and Rituximab in Relapsed or Refractory Hairy Cell Leukmia Variant: A Case Report and Review of Literature / 고신대학교의과대학학술지

Hairy cell leukemia (HCL) is a rare chronic B cell leukemia morphologically characterized by cells with an abundant cytoplasm and hair-like projections that can be found in the peripheral blood and bone marrow. The treatment for HCL is splenectomy or chemotherapy with the purine analogs pentostatin and cladribine. However, patients continue to relapse. Retreatment with the same or alternate purine analogs produces lower response rates and a shorter duration of response. Fludarabine is another purine analog widely used in treating indolent lymphoid cancers, often in combination with rituximab. Here, we report a case of HCL variant in a 60-year-old man who experienced multiple relapses after splenectomy and retreatment with cladribine. The patient was then treated with fludarabine and rituximab combination chemotherapy. After the treatment, he achieved complete remission that continued for 35 months.

Clinical analysis of 24 patients of hairy cell leukemia treated by cladribine / 中华血液学杂志

Objective: To investigate the curative effect of hairy cell leukemia by clatabine. Methods: The clinical data of 24 patients with hairy cell leukemia treated by cladribine from November 2006 to October 2017 were analyzed retrospectively, then the curative effect and adverse drug reaction were analyzed. Results: ① A total of 24 patients including 22 male and 2 female, and the median age was 49.5 years (range 33 to 76) at diagnosis. There were 20 patients with of splenomegaly (4 patients with mild splenomegaly, 4 moderate splenomegaly, and 12 massive splenomegaly), 3 patients with enlargement of lymph nodes, and 1 patients who had undergone splenectomy. Five patients were pancytopenia, 15 were cytopenia in 2 lineages, and 4 patients were cytopenia only in one lineage. The median ratio of HCL cells detected by flow cytometry in bone marrow was 21.79% (0.69%-68.96%). BRAF mutation was detected in 15 patients by first generation or next generation sequencing technology. ② Among 24 patients, 20 were treated with cladribine alone (one course in 19 patients, 2 courses in 1 patient), and 4 patients were treated with cladribine combined with rituximab (one course in 3 patients, 2 courses in 1 patient). Excepting 5 patients whose follow-up time was not reaching 6 months, 19 patients were evaluated for efficacy in 6-12 months after treatment: 9 patients obtained CR, 9 obtained unconfirmed CR (Cru), the other 1 obtained PR, the CR/CRu rate was 94.7%, the overall response rate (ORR) was 100.0%. ③ All the 24 patients appeared 2-4 grade hematological adverse reactions after cladribine treatment, which were mainly grade 3/4 neutropenia (66.67%) and grade 3/4 thrombocytopenia (29.2%). All the adverse reactions were controlled or recovered spontaneously. ④ After the median follow-up time of 15 (3-133) months, no progression, recurrence or death occurred in the patients. Both median OS and PFS were not reached. Conclusion: This study suggests that treatment of HCL with cladribine has high response rate, controllable adverse reactions and the good prognosis.

Clinical analysis of 24 patients of hairy cell leukemia treated by cladribine / 中华血液学杂志

Objective@#To investigate the curative effect of hairy cell leukemia by clatabine. @*Methods@#The clinical data of 24 patients with hairy cell leukemia treated by cladribine from November 2006 to October 2017 were analyzed retrospectively, then the curative effect and adverse drug reaction were analyzed. @*Results@#① A total of 24 patients including 22 male and 2 female, and the median age was 49.5 years (range 33 to 76) at diagnosis. There were 20 patients with of splenomegaly (4 patients with mild splenomegaly, 4 moderate splenomegaly, and 12 massive splenomegaly), 3 patients with enlargement of lymph nodes, and 1 patients who had undergone splenectomy. Five patients were pancytopenia, 15 were cytopenia in 2 lineages, and 4 patients were cytopenia only in one lineage. The median ratio of HCL cells detected by flow cytometry in bone marrow was 21.79% (0.69%-68.96%). BRAF mutation was detected in 15 patients by first generation or next generation sequencing technology. ② Among 24 patients, 20 were treated with cladribine alone (one course in 19 patients, 2 courses in 1 patient), and 4 patients were treated with cladribine combined with rituximab (one course in 3 patients, 2 courses in 1 patient). Excepting 5 patients whose follow-up time was not reaching 6 months, 19 patients were evaluated for efficacy in 6-12 months after treatment: 9 patients obtained CR, 9 obtained unconfirmed CR (Cru), the other 1 obtained PR, the CR/CRu rate was 94.7%, the overall response rate (ORR) was 100.0%. ③ All the 24 patients appeared 2-4 grade hematological adverse reactions after cladribine treatment, which were mainly grade 3/4 neutropenia (66.67%) and grade 3/4 thrombocytopenia (29.2%). All the adverse reactions were controlled or recovered spontaneously. ④ After the median follow-up time of 15 (3-133) months, no progression, recurrence or death occurred in the patients. Both median OS and PFS were not reached. @*Conclusion@#This study suggests that treatment of HCL with cladribine has high response rate, controllable adverse reactions and the good prognosis.

An extremely rare case of concurrent BRAF V600E mutation driven hairy cell leukemia and melanoma: case report and review of literature

BRAF protein is a serine/threonine kinase with 766 amino acids. Approximately 15% of human cancers harbor BRAF mutations as well as other BRAF anomalies (amplifications, fusions). Somatic mutations mainly occur in the catalytic kinase domain (CR3), and the predominant mutation is p.V600E which is the substitution of glutamic acid (E) for valine (V) as result of a mutation at codon 600 of the kinase domain. To our knowledge, the vast majority of the cancers have non-germline BRAF mutations. Here we describe a case of a 60-year-old female with a history of hairy cell leukemia (HCL) who presented with aphasia and forgetfulness. A follow-up Brain CT scan showed three distinct brain lesions which were found to be diagnostic of melanoma (confirmed by immunohistochemistry) with no evidence of a concurrent brain involvement by a B-cell neoplasm. Molecular studies confirmed the same BRAF p.V600E mutation in both malignancies (hairy cell leukemia and melanoma). Thereafter the patient was started on BRAF inhibitor treatment and is now symptom-free after one year of follow up. Having two concurrent malignancies with a shared BRAF mutation is extremely rare and makes this an excellent example of a genomic marker-driven treatment in two histologically and immunophenotypically distinct tumors.

Haematological profile of 21 patients with hairy cell leukaemia in a tertiary care centre of north India.

Background & objectives: Hairy cell leukaemia (HCL) is a B cell neoplasm which constitutes around 2 per cent of all the lymphoid leukaemias. It has a characteristic morphology and immunophenotypic profile. It is important to distinguish HCL from other B cell lymphoproliferative disorders due to availability of different chemotherapeutic agents. This study presents clinical, haematological and immunophenotypic profile of patients with HCL seen over a period of four years in a tertiary care hospital in north India. Methods: Twenty one cases of hairy cell leukaemia were analyzed for their clinical details, haemogram, bone marrow examination and immunophenotypic findings. Results: Age of the patients ranged from 28-76 yr with male predominance. Weakness and fever were commonest presentations. Splenomegaly, hepatomegaly, lymphadenopathy were seen in decreasing order of frequency. Anaemia was noted in all 21 patients, leukopenia in 15 and thrombocytopenia in 19 cases. Fourteen patients were pancytopenic. Bone marrow examination showed typical hairy cells in all cases. Immunophenotyping showed expression of CD19, CD20, CD103, CD25 and CD11c in all cases, while positivity was seen for CD79b in 93.7 per cent, kappa light chain restriction in 60 per cent and lambda in 40 per cent cases. Notably, 20 per cent showed CD10 and 12 per cent showed CD23 expression. Interpretation & conclusions: This study reveals some unusual findings in otherwise classical disease entity, like absence of palpable spleen, presence of lymphadenopathy, normal or elevated leukocyte counts, expression of CD10, which at times could be diagnostically challenging.

V-raf murine sarcoma viral oncogene homolog B (BRAF) mutations in hairy cell leukaemia.

Introduction: Hairy cell leukemia (HCL) is a B-cell non-Hodgkin lymphoma with distinct clinical, morphological and immunophenotypic features; however, there are many other B-cell lymphomas, which closely mimic HCL. Accurate diagnosis of HCL is important as treatment with 2-chloro-2’-deoxyadenosine (cladribine) is associated with >80% chance of complete cure. The recent description of BRAF p.V600E mutations in almost all HCL cases in various studies has not only improved the pathogenetic understanding of this entity but also increased the diagnostic accuracy of this disorder. Aim: The aim of the study was to standardize a molecular test for diagnosis of HCL and compare with standard established morphological, cytochemical and immunophenotypic parameters for HCL diagnosis. Materials and Methods: The incidence of this mutation was sought in 20 patients with either classical HCL or HCL variant (HCLv) by Sanger sequencing and allele-specifi c polymerase chain reaction. BRAF p.V600E mutation was present in all HCL cases and absent in the only HCLv case. Results: A high degree of correlation was noted between the presence of BRAF p.V600E and established diagnostic criteria in 20/20 patients with HCL/ HCLv. Our data supports the observation that this mutation is present in all cases of HCL and is absent in HCLv. Hence, detection of the BRAF p. V600E mutation can be a useful adjunct in the diagnostic algorithm.

Complete recovery of pyoderma gangrenosum after successful treatment of underlying hairy cell leukemia with cladribine

No abstract available.

Hairy-cell leukemia variant: a case report and review of the literatures / 白血病·淋巴瘤

Objective To study the clinical features and differential diagnosis of hairy-cell leukemia variant (HCL-V).Methods A case with HCL-V was reported and the literatures were reviewed.Results The patient had splenomegaly for twenty years and a history of recurrent pulmonary infection.His blood routine test showed a high white blood cell count and abnormal high proportion of lymphocytes.Peripheral smear and bone marrow smear both showed significantly higher proportion of lymphocytes,part of which had soma jagged,prominent nucleoli and villous/hairy cytoplasmic projections.His hairy leukemic cells expressed CD1 1c,CD19,CD20,CD22,and had variable expression of FMC-7,CD103 and lambda,but not CD5,CD23 and CD25.Transmission electron microscope showed many monocytes with villous exist in peripheral blood.Conclusions HCL-V is a rare and an indolent form of a small,mature,B-cell leukemia,based on the clinical,peripheral smear,bone marrow smear,flow cytometric analysis and transmission electron microscopy features,a diagnosis of HCL-V is confirmed.The differential diagnosis should always include splenic marginal zone B-cell lymphoma and HCL-C,because they have different clinical and biological features.

Hairy cell leukemia: a rare case report.

Hairy cell leukemia (HCL) is a rare chronic B-cell leukemia accounting for 2% of all the leukemias and occurs more frequently in the elderly males. The etiology is unknown but possible relationships to radiation exposure, exposure to benzene, to farm animals and to commercial herbicides and pesticides have been identified. Familial predisposition among first degree relatives has been noted. It is characterized by distinctive cytoplasmic “hair like” projections on the cell surface of lymphoid cells, pancytopenia and splenomegaly. We report a rare case of 29 year old female, farm labourer presenting with fever, fatigue and weakness for 1 month. On examination the patient had hepatomegaly, massive splenomegaly and inguinal lymphadenopathy. After peripheral smear examination diagnosis of HCL was made which was confirmed by bone marrow aspiration examination, bone marrow biopsy and immunohistochemistry (IHC).

Cladribine for treatment on hairy cell leukemia: three cases report and literatures review / 白血病·淋巴瘤

Objective To observe clinical response of the cladribine in the treatment of hairy cell leukemia.Methods Three patients were treated with cladribine 10 mg ivgtt for 3 or 5 days.Results Among 3 patients,2 patients achieved complete remission and 1 patient achieved near complete remission.Conclusion Cladribine has high efficacy and a favorable toxicity when adminisered to patients with hairy cell leukemia.

Estafilococcia em paciente com tricoleucemia: relato de caso / Staphylococci in patient with hairy cell leukemia. Case report

JUSTIFICATIVA E OBJETIVOS: A leucemia de células pilosas (LCP) é caracterizada clinicamente por esplenomegalia, pancitopenia e raramente acomete linfonodos. Nos casos mais graves de leucopenia a predisposição às infecções é mais acentuada. Apesar dos métodos diagnósticos para a LCP estarem bem estabelecidos, o objetivo deste estudo foi alertar para o fato de que infecções persistentes podem indicar imunossupressão, devendo portanto, lembrar da LCP como hipótese diagnóstica. RELATO DO CASO: Paciente do sexo masculino, 47 anos, deu entrada no pronto-socorro com quadro clínico de estafilococcia. A cultura do material da punção mostrou a presença de Staphylococcusaureus. Foi realizado mielograma, cujo aspirado mostrou hipocelularidade global, a biópsia concluiu aplasia de medula ósseaque em associação com a imuno-histoquímica mostrou umprocesso linfoproliferativo de células B, que sugeriu continuar ainvestigação. Posteriormente foi realizado a imunofenotipagemem painel proliferativo de sangue periférico com perfil positivopara: CD20**, cadeia leve lambda*, FMC-7, CD19, CD79b,IgM, IgD, CD11c**, CD25, CD200, CD22, CD103, CD123,CD45 e bcl-2, compatível com LCP.CONCLUSÃO: Pacientes com neoplasias hematológicas são acometidos frequentemente por infecções bacterianas na corrente sanguínea, sendo o Staphylococcus aureus amplamente isoladonas hemoculturas.

BACKGROUND AND OBJECTIVES: Hairy cell leukemia(HCL) is characterized clinically by splenomegaly, pancytopenia and the lymphonodes are rarely palpable. In more sever cases of leukopenia, predisposition to infections is more pronounced. Although the diagnostic methods for the HCL are well established, the objective of this study was to draw attention to the fact that persistent infections may indicate immunosuppression, and therefore remember the HCL as a diagnostic hypothesis. CASE REPORT: Male patient, 47 year-old, was admitted to the hospital with clinical staphylococci. Material culture punctures howed the presence of Staphylococcus aureus. Myelogram was performed, which showed global hypocellular aspirate, the bone marrow biopsy concluded for bone marrow aplasia that inassociation with immunohistochemistry showed a lymphoproliferative process of B cell, which suggested keep the investigation. After that was performed a immunophenotyping panel of prolife rative peripheral blood that showed a positive profile for: CD20**, lambda light chain**, FMC7, CD19, CD79b, IgM,IgD, D11c **, CD25, CD200, CD22, CD103, CD123, CD45 and bcl2, consistent with HCL. CONCLUSION: Patients with hematological malignancies are often affected by bacterial infections in the bloodstream, where Staphylococcus aureus was largely isolated in blood cultures.

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.

Relato de caso de leucemia de células pilosas / Case report of hairy cell leukemia

A leucemia de células pilosas (LCP) é um tipo raro de linfoma não Hodgkin de células B. O quadro clínico inclui esplenomegalia, pancitopenia e linfocitose. Estudos de carcinogênese da doença revelam sua associação a agentes químicos agrícolas. O objetivo deste estudo foi o relato de um caso de paciente com LCP, masculino, tratorista, com pancitopenia, lesões de pele, sem esplenomegalia e com marcadores positivos para linfócitos B (CD19, CD20, CD22, CD79b, CD23, Lambda, imunoglobulina M [IgM], CD25 e CD103). Embora a LCP seja uma doença rara, a demora em seu diagnóstico pode levar a sérias complicações e à morte do paciente antes do diagnóstico.

Hairy cell leukemia (HCL) is a rare type of B-cell non-Hodgkin's lymphoma. The clinical symptoms include splenomegaly, pancytopenia, and lymphocytosis. Studies on its carcinogenesis reveal association with exposure to agricultural chemical agents. The objective of this study was to report the case of a male patient, tractor operator, diagnosed with HCL, pancytopenia, cutaneous lesions, without splenomegaly and positive markers for B-cell lymphocytes (CD19, CD20, CD22, CD79b, CD23, Lambda, immunoglobulin M [IgM], CD25 and CD103). Although HCL is a rare disease, late diagnosis may ultimately lead to severe complications and patient's death.

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.