Leukemic reticuloendotheliosis (L.R.E.) was first described as a clinical and pathological entity by Ewald in 1923. LRE is a rare neoplastic disease of the hematopoietic system that is characterized cliniclly by chronic course with an insidious onset. marked splenomegaly with absence of substantial lymphadenopathy and predominant in male and characterized histologically by the presence of circulating abnormal mononuclear cells with many cytoplasmic projections, which have been refered to ??airy cells frequently. Splenectomy appeared to be the most beneficial treatment at present for those patients with massive splenomegaly and hypersplenism and chemotherapy is of little benefit in the treatment in LRE. Recently, the authors had the opportunity to observe a two year and eight old male child at this hospital whose clinical course and pathologid features were consistent with LRE. Splenectomy is performed and discharged with relatively good conditions.
Child ; Cytoplasm ; Drug Therapy ; Hematopoietic System ; Humans ; Hypersplenism ; Leukemia, Hairy Cell* ; Lymphatic Diseases ; Male ; Splenectomy ; Splenomegaly

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.
Bone Marrow ; Cladribine ; Cytoplasm ; Drug Therapy ; Drug Therapy, Combination ; Humans ; Leukemia, B-Cell ; Leukemia, Hairy Cell ; Middle Aged ; Pentostatin ; Recurrence ; Retreatment ; Rituximab ; Splenectomy

Antineoplastic Agents ; administration & dosage ; therapeutic use ; Cladribine ; administration & dosage ; therapeutic use ; Humans ; Leukemia, Hairy Cell ; drug therapy ; Male ; Middle Aged

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

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.
Adult ; Aged ; Antineoplastic Agents/therapeutic use* ; Cladribine/therapeutic use* ; Female ; Humans ; Leukemia, Hairy Cell/drug therapy* ; Male ; Middle Aged ; Retrospective Studies ; Rituximab

Objective: To evaluate the clinical characteristics, treatment response, and outcomes in patients with classical hairy cell leukemia (cHCL) and HCL variant (HCL-V). Methods: This is a retrospective case series study. Between January 2011 and December 2021, clinical data of 30 patients newly with diagnosed HCL at Peking Union Medical College Hospital were analyzed. The main outcome measures include clinical characteristics, treatment efficacy and survival. The Kaplan-Meier method was used for survival analysis. Results: Twenty-one cases of cHCL and 9 cases of HCL-v were included. The median age at diagnosis was 55.5 (range, 30-86) years, with the ratio of male to female 2.75∶1. The main clinical manifestations included fatigue in 11 cases (36.7%), abdominal distension in 7 cases (23.3%), and infection in 4 cases, while 8 cases were asymptomatic. Splenomegaly was reported in 24 cases (80.0%), including 7 (23.3%) with megalosplenia. The white blood cell count, lymphocyte count, and the proportion of peripheral hairy cells in HCL-v group were significantly higher than those in cHCL group, whereas the development of anemia, thrombocytopenia, and monocytopenia in cHCL group was more remarkable than that in HCL-v group (all P<0.05). The BRAF-V600E gene mutation was detected only in cHCL patients (11/14 vs. 0/9, P<0.001). In terms of immunophenotype, the expression of CD25, CD103, CD123 and CD200 in cHCL group (20/20, 20/20, 4/7, 7/17) were all stronger than those in HCL-v group (3/9, 7/9, 0/4, 2/8). Twenty-two patients were treated, of which 13 cases (12 cases of cHCL and 1 case of HCL-v) with cladribine, and 9 cases (4 cHCL and 5 HCL-v) with interferon. Complete remission rate and overall response rate were comparable between cladribine and interferon treatment groups (both P<0.05). The median follow-up time was 31 (range, 1-125) months, and the median overall survival (OS) of the entire group was 125 months. The 5-year OS rate in HCL-v patients represented a trend of inferior (50.0% vs. 95.0%, P=0.207). Conclusions: The clinical features of HCL are unspecific, which includes fatigue, splenomegaly and recurrent infection. The clinical features, immunophenotype, treatment response and prognosis of HCL-v are different from those of cHCL. BRAF-V600E gene mutation is suggested as a key marker for differential diagnosis. Cladribine is recommended as front-line regimen of cHCL patients with satisfactory efficacy and prognosis. Conversely, response and clinical outcome in HCL-v patients still need to be improved.
Humans ; Male ; Female ; Adult ; Middle Aged ; Aged ; Aged, 80 and over ; Leukemia, Hairy Cell/drug therapy* ; Cladribine/therapeutic use* ; Splenomegaly/drug therapy* ; Retrospective Studies ; Proto-Oncogene Proteins B-raf/therapeutic use* ; Prognosis ; Interferons/therapeutic use* ; Antineoplastic Agents/therapeutic use*

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

Aged ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bleomycin ; therapeutic use ; Dacarbazine ; therapeutic use ; Doxorubicin ; therapeutic use ; Hodgkin Disease ; drug therapy ; pathology ; Humans ; Leukemia, Hairy Cell ; drug therapy ; pathology ; surgery ; Male ; Mitoxantrone ; administration & dosage ; Neoplasms, Multiple Primary ; drug therapy ; pathology ; surgery ; Rituximab ; Splenectomy ; Vidarabine ; administration & dosage ; analogs & derivatives ; Vinblastine ; therapeutic use

No abstract available.
Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Female ; Humans ; Immunoglobulin Variable Region/genetics ; Leukemia, Hairy Cell/drug therapy/*genetics/pathology ; Lymphoma, Non-Hodgkin/drug therapy/*genetics/pathology ; MAP Kinase Kinase 1/*genetics ; Male ; Middle Aged ; Mutation ; Polymorphism, Single Nucleotide ; Prednisone/therapeutic use ; Pregnancy ; Proto-Oncogene Proteins B-raf/*genetics ; Real-Time Polymerase Chain Reaction ; Vincristine/therapeutic use