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.

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.

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.