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)

Hairy cell leukaemia is a rare chronic lymphoproliferative disease, characterized by splenomegaly, pancytopenia and recurrent infection. The characteristic 'hairy cells', present in the peripheral blood and bone marrow, are the hallmark of this leukaemia. The disease has a chronic, progressive course, and the majority of patients afflicted by it require therapy. The most common reason to initiate treatment is neutropenia with or without associated infectious complications, or the development of severe thrombocytopenia. Therapeutic options in hairy cell leukaemia include splenectomy, interferon administration, or the use of chemotherapeutic agents such as pentostatin (2'-deoxycoformycin) and 2-chlorodeoxyadenosine. Splenectomy is still indicated in the treatment of young patients with significant splenomegaly and only minimal bone marrow involvement. Interferon treatment induces remission in approximately 90% of patients with hairy cell leukaemia, but complete remission is obtained in only 5-10%. The development of antibodies against interferon was initially considered a major problem, but longer follow-up of patients who developed antibodies has shown that it is transient and does not have a significant impact on the overall response to treatment. Pentostatin induces complete remission in 60-70% of patients and partial remission in 20-40%. 2-Chlorodeoxyadenosine is a very promising drug in the treatment of this rare leukaemia, inducing long-lasting complete remission in approximately 80% of patients. While interferon does not cure the disease, it is possible that a subset of patients treated with pentostatin or 2-chlorodeoxyadenosine are cured. Longer follow-up of these patients will determine whether this is true.
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PMID:Hairy cell leukaemia. 791 37

The quantitative composition of the peripheral blood, enzymatic activity of neutrophils and hypoxic ventilatory drive were determined in people working at the Chernobyl NPP during sojourn for 28 days in the Caucasus mountains at 220 m altitude with a periodical climbing up to 4200 m. At the initial state we found neutropenia, lymphocytosis, an increase in the number of prolymphocytes and big hairy lymphocytes, a decrease in the number of small lymphocytes, fall in activity of enzymes responsible for oxygen-dependent and oxygen-independent mechanisms of bactericidity. The indices of sensitivity to the hypoxic respiration stimulus did not essentially differ from the norm. Staying at altitude promoted normalization of the number of segmento-nuclear neutrophils, lymphocytes (their small population, in particular), an increase in the number of eosinophiles of the peripheral blood. Changes in the activity of myeloperoxidase, NADP-oxidase and cationic proteins in the neutrophils were observed. A considerable increase of the ventilatory drive to the hypoxic respiration stimulus was determined. Accentuation of fermentative transformations proved to be closely related to the ventilatory response to hypoxia: weak activation of NADP-oxidase and a decrease of myeloperoxidase activity were observed in people with initially low respiration reactivity, while a considerable increase in activity of these enzymes and in content of cationic proteins was observed in people with high respiration reactivity. Individual peculiarities of the reaction to staying at altitude should be taken into account when developing particular methods of immune correction.
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PMID:[Respiratory reactivity and parameters of nonspecific immunity in workers of the Chernobyl nuclear power plant during adaptation to mountain climate]. 824 14

A rare aleukemic myeloaplastic presentation of hairy cell leukemia (HCL) is described. The patient was observed for 11 years and presented a clinical picture suggestive of pure white cell aplasia for seven years. Hairy cells (HC) were first discovered in the bone marrow, then occasionally in the blood during the last four years. A brief course of low-dose alpha interferon promptly induced prolonged remission of neutropenia. In our opinion, this case, as few others described till now, should be considered a rare HCL variant, an understanding of which offers an important clinical opportunity for investigating the myeloid inhibitory activity of hairy cells.
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PMID:Selective myeloid aplasia: a long-lasting presentation of an unusual hairy cell leukemia variant? 829 56

Cladribine is a newly developed antimetabolite with promising activity in lymphoproliferative disorders. Recent pharmacokinetics investigations have suggested that there is a relationship between its plasma area under the concentration versus time curve (AUC) and the degree of neutropenia posttreatment as well as the therapeutic outcome in hairy-cell leukemia. To enable a simple estimation of the plasma AUC, a limited sampling strategy was developed. Stepwise linear regression was used to determine which were the most important data points for estimation of the plasma AUC after 2-h i.v. infusion, s.c. injection (5 mg/m2), and oral administration (10 mg/m2) in 27 patients. The most important data points after i.v. infusion in 12 patients were 1, 4, and 24 h, in order of importance. The AUC could be estimated as 2.9081 x C1h + 5.1851 x C4h + 20.3265 x C24h. The accuracy and precision (mean value +/- SD for the determined/estimated AUC was 0.99 +/- 0.053) of the model could not be increased by the addition of more data points. A somewhat lower accuracy and precision (0.96 +/- 0.089) was seen with the 2-, 4-, and 24-h data points. These were used to test the regression technique prospectively for the estimation of the AUC after i.v. administration in another set of 10 patients. The accuracy and precision of the estimation of the AUC was similar in this group (1.01 +/- 0.109). In all, 11 patients were treated orally (10 mg/m2) and 10 patients were treated by s.c. injection (5 mg/m2). The most important data points for estimation of the AUC were 2.5, 24, and 0.5 h after oral administration (AUC = 0.8630 x C0.5h + 4.2337 x C2.5h + 45.4364 x C24h) and 9, 1, and 16 h after s.c. injection (AUC = 1.8821 x C1h + 16.4256 x C9h + 25.4518 x C16h). The accuracy and precision were 1.01 +/- 0.064 after oral dosing and 0.99 +/- 0.11 after s.c. injection. The derived mathematical models are reliable for estimation of the plasma AUC of cladribine after 2-h i.v. infusion, oral administration, and s.c. injection.
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PMID:A limited sampling strategy for estimation of the cladribine plasma area under the concentration versus time curve after intermittent i.v. infusion, s.c. injection, and oral administration. 882 95

Cytokines are involved in hematopoiesis by regulating proliferation, differentiation and cellular functions of various lineages of hematopoietic cells. There is an increasing range of clinical conditions in which cytokines are involved as therapeutic agents. One of the most advanced and successful applications is the stimulation of hematopoiesis by the colony stimulating factors (GM-CSF and G-CSF) and erythropoietin. Hematopoietic growth factors are effective in accelerating recovery from neutropenia after chemotherapy and bone marrow transplantation and in reducing incidence of infections. Interferon alpha (IFN-alpha) proved a useful therapeutic agent for chronic myelogenous and hairy cell leukemias as well as for multiple myeloma and non-Hodgkin's lymphoma. Interleukin 2 is the only cytokine apart from IFN-alpha accepted as antineoplastic agent. It may be useful as adjuvant therapy in the hematological malignancies. It may be supposed that in the near future new recombinant cytokines will be introduced in the treatment of blood diseases.
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PMID:Cytokines in the treatment of hematological disorders: recent progress and perspectives. 887 63

Between January 1991 and January 1994, 40 patients with hairy-cell leukemia (HCL), 30 males and 10 females, with a median age of 54 years, were treated with a single course of 2-chlorodeoxyadenosine (2-CdA) at a dose of 0.1 mg/kg/day continuous infusion for 7 days. Thirteen patients were untreated and 27 had previously received alpha-interferon. Thirty out of 40 patients (75%) achieved complete remission (CR) and 10 (25%) partial remission (PR). The median follow-up duration for patients in CR has been 48 months (range 30-66). Five of the complete responders (17%) relapsed at 12, 24, 26, 30 and 36 months after treatment as documented by the increase of hairy cells (Hc) in the bone marrow and two of them, who were retreated with 2-CdA after showing an initial impairment of peripheral blood values, obtained a second CR. The remaining three relapsed patients were never retreated and still show normal peripheral counts after 30, 38 and 40 months. Twelve of the continuous complete responder patients are still in CR after more than 5 years. In contrast, 8 out of 10 partial responders progressed after 8-36 months and all of them were retreated with 2-CdA at a dose of 0.15 mg/kg/day for 5 days i.v. Four of them (50%) achieved a CR, three a better PR and one patient died 6 months after the second 2-CdA course because of infectious complications. Two additional patients, both in CR, died after 28 and 37 months because of a second neoplasm. Toxic side-effects consisted of febrile episodes recorded in 16 patients: in seven of them, fever lasted only 24-48 h after the end of treatment and was apparently not infection-related. In the remaining nine patients, showing in addition severe neutropenia (neutrophils less than 1.0 x 10(9)/l), fever was related to bacterial infection requiring systemic antibiotics in all of them and G-CSF in three cases. In conclusion, 2-CdA induces a very high proportion of complete and long-lasting remissions in patients with HCL. In a number of cases relapse at bone marrow level may not affect peripheral blood values for prolonged time. However, in those patients with initial pancytopenia a retreatment with 2-CdA is still effective in inducing a durable second CR.
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PMID:Long-lasting complete remission in patients with hairy cell leukemia treated with 2-CdA: a 5-year survey. 918 Feb 83

A patient with hairy-cell leukaemia was treated with granulocyte colony stimulating factor lenograstim (Granocyte) 300 micrograms daily by subcutaneous injections. His pre-existing neutropenia remitted and the therapy was continued for a total of 4 months. When the therapy was discontinued the neutropenia returned. There was no evidence that the growth factor itself had any disease modifying activity.
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PMID:The place of myeloid growth factors in the treatment of hairy-cell leukaemia. 921 56

A retrospective study of 37 patients with haematological malignancy (21 acute myeloid leukaemia, 11 acute lymphoid leukaemia, two lymphoma, two hairy cell leukaemia, one Hodgkin's disease) and histologically documented mucormycosis was conducted to evaluate the clinical characteristics and ascertain the factors which influenced the outcome from mycotic infection. Patients were admitted to 18 haematology divisions in tertiary care or university hospitals in Italy between 1987 and 1995. Fever, thoracic pain, dyspnoea and cough were the most frequent presenting symptoms. At the onset, 89% patients were neutropenic (neutrophil counts < 0.5 x 10(9)/l) with a median duration of previous neutropenia of 14 d (range 6-60). The most frequent sites of infection were lungs (81%), CNS (27%), sinus (16%), liver (16%) and orbital space (10%). Only three patients were asymptomatic. A correct in vivo diagnosis was made in only 13 (35%) patients. When performed, thoracic and cranial CT scan were the most useful diagnostic investigations. Despite the fact that 26 febrile patients were treated with empirical antifungal treatment, 28 of the 37 patients (76%) died from fungal infection at a median time of 17 d from the onset of clinical symptoms. Nine patients were cured by antifungal therapy plus, in five cases, radical surgery procedures. An analysis of factors influencing outcome demonstrated that the resolution of chemotherapy-induced neutropenia and prolonged treatment with amphotericin B and, if feasible, radical surgical debridement treatment, were significantly correlated with recovery from infection. Mucormycosis, a rare filamentous fungal infection that occurs most frequently in neutropenic acute leukaemia patients, is characterized by a high mortality rate. Extensive and aggressive diagnostic and therapeutic procedures are essential to improve the prognosis in these patients.
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PMID:Mucormycosis in patients with haematological malignancies: a retrospective clinical study of 37 cases. GIMEMA Infection Program (Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto). 937 50

Hairy cell leukaemia (HCL) is a rare lymphoproliferative disorder associated with pancytopenia, splenomegaly and the presence of typical hairy B lymphocytes in the bone marrow and/or peripheral blood. The most significant complication relates to opportunistic infections that arise as a consequence of neutropenia and monocytopenia. HCL is occasionally associated with systemic autoimmune disorders including polyarteritis nodosa and rheumatoid disease. Secondary autoimmune haemolytic anaemia (AIHA) appears to be rare. We report on two cases of HCL complicated by fatal cold anti-i AIHA. Fulminant haemolysis causing death is rare in cold AIHA and only a few individual cases have been reported, none having anti-i specificity.
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PMID:Fatal cold anti-i autoimmune haemolytic anaemia complicating hairy cell leukaemia. 1088 17

We describe a case of successfully treated multifocal pulmonary Rhizomucor pusillus, a condition which has previously been universally fatal. A 77 year-old man had a background of chronic neutropenia due to hairy-cell leukemia, splenectomy, corticosteroid therapy and an obstructing left ureteric transitional-cell carcinoma. He was successfully treated with 3 months of high-dose liposomal amphotericin B and 7 months of granulocyte-macrophage colony-stimulating factor. Treatment was complicated by mild reversible deterioration of renal function. There was a near complete radiological response to the therapy at 6 months and the patient remains well 20 months following diagnosis of R. pusillus and 13 months following cessation of treatment.
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PMID:Cure of pulmonary Rhizomucor pusillus infection in a patient with hairy-cell leukemia: role of liposomal amphotericin B and GM-CSF. 1191 24


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