Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Daily administration of 2-chlorodeoxyadenosine (Cladribine, CDA) is a standard treatment for hairy cell leukemia, but may cause severe neutropenia and neutropenic fever. This trial compared toxicity and efficacy of weekly versus daily CDA administration. One hundred patients were randomized to receive standard (CDA 0.14 mg/kg/day day 1-5 [Arm A]) or experimental treatment (CDA 0.14 mg/kg/day once weekly for 5 weeks [Arm B]). The primary endpoint was average leukocyte count within 6 weeks from randomization. Secondary endpoints included response rates, other acute hematotoxicity, acute infection rate, hospital admission, remission duration, event-free, and overall survival. There was no significant difference in average leukocyte count. Response rate (complete + partial remission) at week 10 was 78% (95% confidence interval (CI) 64-88%) in Arm A and 68% (95% CI 54-80%) in Arm B (p = 0.13). Best response rates during follow-up were identical (86%) in both arms. No significant difference was found in the rate of grade 3+4 leukocytopenia (94%vs. 84%), grade 3+4 neutropenia (90%vs. 80%), acute infection (44%vs. 40%), hospitalization (38%vs. 34%), and erythrocyte support (22%vs. 30%) within 10 weeks. Overall, these findings indicate that there are no apparent advantages in toxicity and efficacy by giving CDA weekly rather than daily.
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PMID:Randomized trial of daily versus weekly administration of 2-chlorodeoxyadenosine in patients with hairy cell leukemia: a multicenter phase III trial (SAKK 32/98). 1967 71

Cladribine is approved to be used in 24-hour continuous infusion for the treatment of low-grade lymphoma by the Ministry of Health, Labor and Welfare in Japan. Pharmacokinetic studies showed that the antitumor activity of cladribine by 2-hour infusion should be comparable to that given by continuous infusion. The safety and anti-tumor activity of short infusion of cladribine was shown in hairy cell leukemia, chronic lymphocytic leukemia and low-grade non-Hodgkin's lymphoma in Europe. We therefore underwent a pilot study to confirm the safety and efficacy of cladribine given by 2-hour infusion for Japanese patients with relapsed or refractory indolent B-cell lymphoma. Cladribine at a dose of 0.09 mg/kg was administered in 2-hour intravenous infusion for 5 consecutive days. The treatment was repeated at a 28-day interval for at least 2 cycles, and its efficacy and toxicity were investigated. Fourteen patients were entered into this study. Eight patients (57%) responded to cladribine, including 2 (14%) complete response (CR) and 6 (43%) partial response (PR). The median duration of response was 20+ and 21+ months for CR, and 12 months ranging from 3 to 34 months for PR, respectively. Grade 3 or 4 neutropenia and lymphocytopenia occurred in 43% and 71% of patients, respectively, but there was no febrile neutropenia or opportunistic infection associated with cladribine treatment. No other adverse events greater than grade 3 were encountered. The tumor response and degree of toxicity were comparable with those observed in cladribine treatment given by continuous infusion at a same dose. Cladribine can be administered in 2-hour infusion in an outpatient clinic and is therefore quite convenient for patients.
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PMID:Cladribine treatment in two-hour intravenous infusion for previously-treated low grade B-cell lymphoma: a pilot study. 1990 11

Hairy cell leukemia (HCL) is a rare, chronic, B-cell, lymphoproliferative disorder. Treatment has been revolutionized by the advent of interferon (IFN)-alpha and purine analogs (PA). First-line therapy with PA yields complete response rates of 75-100%, with many long-lasting remissions. In the event of profound neutropenia and/or infectious complications, a short sequence of IFN-alpha may precede PA treatment. Because of the excellent results achieved with PA therapy, the potential role of rituximab (an anti-CD20 monoclonal antibody that is highly effective against most B-cell lymphomas) in HCL has yet to be elucidated. Six HCL cases treated with rituximab are reported herein with a view to elucidating the potential role of the drug in HCL. The indications essentially consist of relapsing or refractory disease, avoiding the cumulative toxicity of PA, consolidation therapy in order to eradicate minimal residual disease, and first-line therapy for patients with contraindications to PA and IFN-alpha.
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PMID:Which role for rituximab in hairy cell leukemia? Reflections on six cases. 2005 82

Severe pandemic influenza A virus (H1N1) infection is associated with risk factors such as pregnancy, obesity and immunosuppression. Immunocompromised patients are at increased risk of more severe or prolonged infection. We report a case of a hairy cell leukemia patient with H1N1 pneumonia which caused severe and prolonged illness. H1N1 virus pneumonia with meticillin-resistant Staphilococcus Aerues (MRSA) coinfection causing Acute Lung Injury (ALI) was treated with a double-dose of osentamyvir, a high dose of teicoplanin and a low dose of corticosteroids. Haematological findings included leucopenia, neutropenia, lymphopenia, reduction of gamma-globulins and natural killer (NK) cells. Reduction of NK and gamma-globulins may explain the development of severe illness and the prolonged illness Neutropenia may explain the MRSA co-infection. Lymphopenia is directly associated with virus action and is considered to be a marker of the swine influenza in adults.
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PMID:Influenza A H1N1 pneumonia in a patient with hairy-cell leukemia. 2094 77

We describe a 69-year-old male patient who was referred for the investigation of long-lasting fever, anemia and neutropenia. Hairy cell leukemia was diagnosed and treated successfully. However, fever persisted despite thorough investigation and use of broad-spectrum antibiotics. Four months after the initial diagnosis, the patient underwent explorative laparotomy and splenectomy. Spleen biopsy revealed multiple necrotizing mycobacterial granulomata while the patient's fever disappeared permanently. Isolated splenic mycobacterial disease is very rare. This case report emphasizes that investigation of chronic fever in hairy cell leukemia requires a high level of clinical suspicion. Early diagnostic procedures for evidence of atypical mycobacterial infection should be considered. When everything else fails, surgery can be helpful in selected cases.
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PMID:Isolated Splenic Mycobacterial Disease: A Cause of Persistent Fever in a Hairy Cell Leukemia Patient. 2106 Jun 95

The natural history of hairy cell leukemia (HCL) includes frequent and potentially life-threatening infections. Prior to the development of effective therapy, the incidence in patients followed for several years was as high as 60%, with infection as a prime cause of death in patients. Studies of the immune system of patients with HCL identified several potential reasons, including profound neutropenia and monocytopenia. In addition, treatment including chemotherapy and splenectomy further compromised the immune system. The success of new therapies has changed the frequency and severity of infections in patients with HCL. During the initial phase of treatment, however, infection risk remains high, with incidence ranging from 30 to 50%. Attempts to ameliorate the risk with growth factors in conjunction with treatment have not been successful, but lower doses of drugs and/or combination therapy have been tried with reported success. In the majority of patients, successful therapy results in normalization of the neutrophil count and marked reduction in the severity and frequency of infections. Interestingly, after purine nucleoside treatment, there is profound depression of CD4+ cells without development of the opportunistic infections seen with patients with human immunodeficiency virus (HIV). Studies to reduce morbidity and mortality should focus on initial induction regimens, as well as confirming the long-term benefit of treatment on risk of infection.
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PMID:Infectious complications in hairy cell leukemia. 2150 85

Hairy cell leukaemia (HCL) is a rare, clonal, chronic lymphoproliferative disorder commonly seen in males in the middle years of life. Pancytopaenia with moderate to massive splenomegaly is the most common clinical presentation. Diagnosis is made on detecting the lymphocytes with abundant cytoplasm which spread into hair-like processes on peripheral blood and bone marrow smears, thus giving the name, "hairy cell leukaemia". The bone marrow aspirate is frequently a dry tap. The trephine biopsy has the characteristic features of a honey comb appearance and flow cytometry is typically CD103, CD25, FMC7, CD11c, gamma or kappa light chain positive with the classic B lymphocyte markers CD19, CD20, CD79a. Purine analogues followed by granulocyte-colony stimulating factor (G-CSF) to manage the febrile neutropenia is currently the treatment of choice. A 10 year disease free survival is recorded with these management strategies. Experimental use of anti CD20 and CD22 has also shown promising results in the treatment of this disease. We report four cases of HCL diagnosed in a span of two years at the Royal Hospital, Muscat, Oman.
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PMID:Hairy Cell Leukaemia in Oman: Four cases. 2174 80

Treatment of hairy cell leukemia (HCL) with cladribine induces durable remissions. Common toxicities are myelosuppression and immunosuppression with low counts of CD4 + T cells. Skin rash (SR) is seldom described. We collected clinical and laboratory data of 35 patients with HCL treated in Hadassah between January 1999 and February 2010, in order to evaluate the frequency and characteristics of SR after treatment with cladribine. We found a high frequency of SR in our group of patients (18/35 patients, 51%), mostly related to febrile neutropenia and concomitant treatment with penicillins/trimethoprim-sulfamethoxazole (TMP-SMZ). The lymphocyte count was low in all patients with SR. We conclude that patients with HCL treated with cladribine have an increased rate of drug hypersensitivity, possibly due to T-cell imbalance induced by cladribine. Since TMP-SMZ and penicillins are related to SR in most cases and are important in the management of patients with HCL, a desensitization protocol should be considered. Rechallenge may be safe after immune reconstitution.
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PMID:High incidence of skin rash in patients with hairy cell leukemia treated with cladribine. 2203 15

Infectious complications remain a significant issue in the care of patients with hematologic malignancies. Inherent immune defects related to the primary disease process are present in patients with disorders such as chronic lymphocytic leukemia, multiple myeloma, hairy cell leukemia, and Hodgkin lymphoma. Therapy-related immunosuppression is also commonplace in these patients. This includes not only treatment-related neutropenia, but also defects in cell-mediated immunity, such as those that occur with purine analog therapy. In this chapter, we will review the pathogenesis of infection in these disorders, as well as the spectrum of infectious complications seen and suggested strategies for the prevention of infection.
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PMID:Infections in patients with leukemia and lymphoma. 2470 30

A 47-year-old woman with pancytopenia, excessive systemic lymphadenopathy and splenomegaly was referred to our hospital. The peripheral blood (PB) smear findings indicated neutropenia with lymphoid cells exhibiting hairy projections, while the histological findings of the cervical lymph node (LN) suggested hairy cell leukemia (HCL). In addition, the BRAF V600E mutation was detected, and the immunoglobulin gene rearrangement patterns were identical in both the cervical LN and PB specimens. Based on these findings, we diagnosed the patient with systemic lymphadenopathy due to HCL. This is the first report of a BRAF mutation detected in both the PB and LN at the onset of HCL.
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PMID:Hairy Cell Leukemia with Systemic Lymphadenopathy: Detection of BRAF Mutations in Both Lymph Node and Peripheral Blood Specimens. 2602 95


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