ABSTRACT
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.
Subject(s)
Humans , Male , Adult , Middle Aged , Aged , Leukemia, Hairy Cell/therapy , Cladribine/therapeutic useABSTRACT
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.
Subject(s)
Child , Female , Humans , Airway Obstruction , Biopsy , Cladribine , Cytarabine , Drug Therapy , Dyspnea , Emergencies , Estrogens, Conjugated (USP) , Histiocytosis, Sinus , Lymph Nodes , Lymphatic Diseases , Methotrexate , Neck , Prednisone , Radiotherapy , Remission, Spontaneous , Trachea , Vinblastine , VincristineABSTRACT
The definition of refractory Langerhans cells histiocytosis (LCH) is risk organs (bone marrow, liver, spleen)involvement and the first-line treatment failure.It has a very poor prognosis.The survival depends on timely and effective salvage treatment.Currently the treatment for refractory LCH includes isolated 2-chlorodeoxyadenosine, 2-chlorodeoxyadenosine combined with high-dose cytarabine, indomethacin, hematopoietic stem cell transplantation and targeted therapy.In this paper, the advances in treatment of the refractory LCH in children were reviewed.
ABSTRACT
Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder that is characterized by cytopenia, splenomegaly, and mononuclear cells displaying cytoplasmic projections. We experienced a case of hairy cell leukemia that developed in a 38-year-old man. He showed marked splenomegaly without palpable lymphoadenopathy. A complete blood cell count revealed leukopenia (3300/microliter with 63% of lymphocyte) and the peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow smear revealed abnormal lymphocytes and severe myelofibrosis. Tartrate-resistant acid phosphatase reactivity was strongly positive in the hairy cells. The immunophenotyping results of lymphoid cells were CD5 (-), CD10 (-), CD19 (+), CD25 (+), CD103 (+), CD20 (+), lambda (+). The patient was treated with 2-Chlorodeoxyadenosine at a daily dose of 0.1mg/Kg by a continuous intravenous infusion for 7 days. The patient achieved complete remission.
Subject(s)
Adult , Humans , Acid Phosphatase , B-Lymphocytes , Blood Cell Count , Bone Marrow , Cladribine , Cytoplasm , Immunophenotyping , Infusions, Intravenous , Leukemia, Hairy Cell , Leukopenia , Lymphocytes , Lymphoproliferative Disorders , Primary Myelofibrosis , SplenomegalyABSTRACT
PURPOSE: Langerhans cell histiocytosis (LCH) is a disorder characterized by the proliferation of activated Langerhans cells. Although current therapies are very effective at inducing remission, multiple recurrences and long-term sequelae are common for young patients. For this reason, more effective therapies based on the pathogenesis of LCH are needed. We investigated the use of 2-chlorodeoxyadenosine (2-CdA), a purine analogue with an antiproliferative effect on histiocytes and lymphocytes, in patients with recurrent or refractory LCH. METHODS: Four children with recurrent or refractory LCH received 2-CdA (5~7 mg/m2/day for 5 days, given as a 24-hr continuous infusion and repeated every 21~28 days for 5~7 courses). RESULTS: All four patients had multiorgan involvement, and were heavily pretreated. Of the two children with recurrent diseases, one had complete response and the other showed no active disease except for the remaining diabetes insipidus. Two infants who showed poor early response to previous combination chemotherapy also responded poorly: partial response in one, and progressive disease resulting in death in the other. Toxicity consisted mainly of myelosuppression, but significant infections did not occur. The peripheral neuropathy was not seen. CONCLUSION: 2-CdA, tolerable in children without significant side effects, might be effective for the treatment of recurrent LCH in children. However, the efficacy in infants with multi-system, refractory diseases needs further study. The feasibility of 2-CdA treatment as the first-line therapy for high-risk diseases, and the possibility of combination with other agents needs to be addressed in the future.
Subject(s)
Child , Humans , Infant , Cladribine , Diabetes Insipidus , Drug Therapy, Combination , Histiocytes , Histiocytosis, Langerhans-Cell , Langerhans Cells , Lymphocytes , Peripheral Nervous System Diseases , RecurrenceABSTRACT
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.
Subject(s)
Humans , B-Lymphocytes , Cladribine , Cytoplasm , Diagnosis , Infusions, Intravenous , Leukemia, Hairy Cell , Lymphoproliferative Disorders , Pentostatin , Prognosis , SplenomegalyABSTRACT
Waldenstrom's macroglobulinemia is a low- grade lymphoproliferative disorder with monoclonal IgM protein. It is characterized by normocytic, normochromic anemia and lymphoplasmacytic marrow infiltration. Chemotherapy with alkylating agents and steroids has been the standard therapy for patients with symptomatic macroglobulinemia. The purine nucleoside analogues, either alone or in combination with other chemotherapeutic agents are increasingly used, and approximately 40% of patients who have received prior therapy with alkylating agents responded. We experienced a case of Waldenstrom's macroglobulinemia suc-cessfully treated with three courses of cladribine, who had previously received unsuccessful therapy using an alkylating agent, steroid and plasmapheresis. Treatment was well tolerated except for frequent upper respiratory infections with severe pancytopenia. A marked and sustained bone marrow suppression occurred in this patient but resolved in three months without any severe infection.