Humans ; Leukemia, Hairy Cell/diagnosis* ; Diagnosis, Differential

Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder characterized by cytopenia, splenomegaly and mononuclear cells displaying cytoplasmic projections. Diagnosis is based on the distinctive hairy cell morphology and immunological profile. In the last 10 to 15 years the prognosis of patients with HCL has improved considerably following the use of purine analogues such as deoxycoformycin and 2-chlorodeoxyadenosine (2-CdA). We report 3 patients with HCL who were treated with 2-CdA at a daily dosage of 0.1mg/kg by continuous intravenous infusion for 7 days. After 1 or 2 courses of treatment, all patients achieved complete remission and are still alive in disease-free status.
B-Lymphocytes ; Cladribine* ; Cytoplasm ; Diagnosis ; Humans ; Infusions, Intravenous ; Leukemia, Hairy Cell* ; Lymphoproliferative Disorders ; Pentostatin ; Prognosis ; Splenomegaly

Hairy cell leukemia-variant (HCL-variant) is a rare B-cell disorder of hairy cell leukemia cases. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. Diagnosis is based on the distinctive hairy cell morphology and immunophenotype. Cells from hairy cell leukemia (HCL) and HCL-variant have a distinct immunophenotype which is of a mature but not terminally differentiated activated B-cell. We experienced a case of HCL- variant in a 50-year-old man with marked splenomegaly and skin eruptions. Peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow was easily aspirated and showed hypercellularity and diffuse interstitial infiltration of hairy cells. Tartrate-resistant acid phosphatase (TRAP) reactivity was negative in the hairy cells. Immunophenotyping results of lymphoid cells were CD2 (-), CD3 (-), CD5 (-), CD7 (-), CD10 (-), CD19 (++), CD20 (+++), sKappa (-), sLambda (+), CD23 (-), CD25 (-), FMC7 (+) and CD103 (++).
Acid Phosphatase ; B-Lymphocytes ; Bone Marrow ; Cytoplasm ; Diagnosis ; Humans ; Immunophenotyping ; Leukemia, Hairy Cell* ; Lymphocytes ; Lymphocytosis ; Middle Aged ; Skin ; Splenomegaly

We experienced a case of atypical hairy cell leukemia in a 42-year-old woman. She showed marked splenomegaly without palpable lymphadenopathy. Complete blood cell count revealed leukocytosis at 44,000/micro L with lymphocytes 74% and peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow was easily aspirated and also revealed the abnormal lymphocytes in up to 95%. Tartrate-resistant acid phosphatase reactivity was negative in the hairy cells. Immunophenotyping results of lymphoid cells were CD5(-), CD7(-), CD10(-), CD19(+), and HLA-DR(+). She was treated with an adenosine analogue, fludarabine at a daily dose of 30mg/m2 for 5 consecutive days, every four weeks. Immediately after treatment, the size of the spleen was normalized. Correct diagnosis was difficult due to insufficient laboratory and pathologic data. The differential diagnosis of mature B-cell neoplasms with cytoplasmic projections in patients with splenomegaly includes hairy cell leukemia and splenic lymphoma with villous lymphocytes. We herein described the present case with a brief review of the literature.
Acid Phosphatase ; Adenosine ; Adult ; B-Lymphocytes ; Blood Cell Count ; Bone Marrow ; Cytoplasm ; Diagnosis ; Diagnosis, Differential ; Female ; Humans ; Immunophenotyping ; Leukemia, Hairy Cell* ; Leukocytosis ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Spleen ; Splenomegaly

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.
Annexin A1 ; Bone Marrow ; Diagnosis ; Humans ; Korea ; Leukemia, Hairy Cell ; Lymphatic Diseases ; Lymphoma ; Lymphoma, B-Cell ; Prognosis ; Sensitivity and Specificity ; Splenectomy ; Splenomegaly

No abstract available.
Adult ; Antineoplastic Agents/*therapeutic use ; Biopsy ; Cladribine/*therapeutic use ; Humans ; Leukemia, Hairy Cell/complications/diagnosis/*drug therapy ; Male ; Pyoderma Gangrenosum/diagnosis/*etiology ; Remission Induction ; Time Factors ; Treatment Outcome

One case of hairy cell leukemia with survival for 10 years was reported. The patient received Interferon-alpha(2b) treatment continuously for more than 9 years and intravenous administration of fludarabine for 2 courses of treatment in the later period, and died due to complication of severer infection in thd end. The manifestation, diagnosis and treatment of hairy cell leukemia were discussed.
Aged ; Humans ; Immunophenotyping ; Interferon-alpha ; therapeutic use ; Leukemia, Hairy Cell ; diagnosis ; drug therapy ; mortality ; Male ; Recombinant Proteins ; Vidarabine ; analogs & derivatives ; therapeutic use

OBJECTIVETo explore the feasibility and diagnostic implication of BRAF V600E mutation identified by high-resolution melting (HRM) assay in patients with hairy cell leukemia (HCL).

METHODSThe V600E mutation of BRAF exon 15 in four HCL patients were detected by HRM assay and patients' clinical data were retrospectively analyzed.

RESULTSAll four HCL patients were positive for the BRAF V600E mutation, which were identical to the results of DNA sequencing.

CONCLUSIONThe HRM assay for BRAF V600E mutation provides a useful tool to aid the laboratory diagnosis of HCL with easy operability, accuracy, and low cost.

Adult ; Aged ; DNA Mutational Analysis ; Female ; Humans ; Leukemia, Hairy Cell ; diagnosis ; genetics ; Male ; Middle Aged ; Mutation ; Polymerase Chain Reaction ; methods ; Proto-Oncogene Proteins B-raf ; genetics

No abstract available.
Antineoplastic Agents/therapeutic use ; Bone Marrow Cells/pathology ; Cladribine/therapeutic use ; DNA Mutational Analysis ; Female ; Humans ; Immunophenotyping ; Leukemia, Hairy Cell/*diagnosis/drug therapy/*genetics ; Middle Aged ; *Mutation ; Proto-Oncogene Proteins B-raf/*genetics ; Reticulin/metabolism

Coxiella burnetii(C. burneii)was first recognized as the agent of Q fever in 1937. Q fever is an acute self-limited febrile illness. However, it manifests with several clinical symptoms depending upon the organs that are involved. The association of C. burnetii with human neoplasia has been rarely reported. We prospectively studied the 55 patients with fever of unknown origin, pneumonia, hepatosplenomegaly, lymphadenopathy, leukemia, lymphoma, and immunodeficiency and 14 persons who contacted the Q fever patients. The patient's sera were tested for antibodies specific for C. burnetii, using indirct fluorescent antibody techniques (IFA). 1) We serologically confirmed 23 C. burnetii infection. The 23 children with Q fever ranged in age from 0 to 15 years, with mean age of 4 years 11 months. Seventeen were boys and 6 were girls. 2) Characteristic symptoms and signs were fever (9/12 cases), rash (8/14 cases), hepatosplenomegaly (8/8 cases)and lymphadenopathy (14/27 cases). Five cases among 14 asymptomatic cases who contacted Q fever patients showed positive IFA test. One suffered from irregular uterine contraction, 4 weeks after contact with a Q fever patient. 3) There were no history of exposure to domestic animal carriers or contaminated dust, or drinking raw milk except one family. Three attending doctors and her father infected by a patient with Q fever. These suggested the person to person transmission of Q fever in a family and house staffs infected by a patient of Q fever. 4) Q fever (9 cases), acute lymphoblastic leukemia (2 cases), acute myelomonocytic leukemia (1 case), hairy cell leukemia (1case), Kawasaki disease (4 cases) and congenital dyserythropoietic anemia (1 case) showed positive IFA test. 5) Of 9 cases who suffered from lnly Q fever, 7 cases were confirmed hairy cell formation in their peripheral blood. One case was diagnosed as hairy cell leukemia after bone marrow study. Of 7 cases who showed hairy cells, all had hepatomegaly, 6 cases had lymphedenopathy and 5 cases showed splenomegaly. All except 1 case who was not followed cured after treatment. 6) We treated Q fever patients with rifampin and/or ciprofloxacin, and/or tetracyclin (over 8 year-old of age)for 2-4 weeks. One 25 month-old patient with hairy cell leukemia was treated with rifampin, ciprofloxacin and tetracyclin for 4 weeks, and rifampin for 8 months. A pregnant patient was administered with rifampin, and treated with rifampin and ciprofloxacin after delivery. We gave rifampin in one nweborn baby. In conclusion, we suggest that Q fever should be considered in the differential diagnosis of patients with FUO, hepatosplenomegaly and/or immunodeficiency.
Anemia, Dyserythropoietic, Congenital ; Animals, Domestic ; Antibodies ; Bone Marrow ; Child ; Child, Preschool ; Ciprofloxacin ; Coxiella burnetii* ; Coxiella* ; Diagnosis, Differential ; Drinking ; Dust ; Exanthema ; Fathers ; Female ; Fever ; Fever of Unknown Origin ; Fluorescent Antibody Technique ; Hepatomegaly ; Humans ; Internship and Residency ; Leukemia ; Leukemia, Hairy Cell ; Leukemia, Myelomonocytic, Acute ; Lymphatic Diseases ; Lymphoma ; Milk ; Mucocutaneous Lymph Node Syndrome ; Pneumonia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prospective Studies ; Q Fever* ; Rifampin ; Splenomegaly ; Uterine Contraction