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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of Wegener granulomatosis of the breast are reported. In both cases, this rare disease was detected on mammographic examination and was diagnosed for the first time by a fine-needle aspiration biopsy of the breast. The mammographic findings can lead to misinterpretations and can be confused with advanced mammary carcinoma or the alterations caused by lymphoblastoma, leukemia, or Hodgkin disease. The total regression of the tumorlike lesions in the breast, evidence of involvement of the lung with only slight clinical symptoms, and a very protracted course of the disease were remarkable in both cases.
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PMID:Wegener granulomatosis of the breast. 396 52

Pulmonary alveolar proteinosis is a rare disease with varied radiographic and clinical manifestations. Thirteen patients are presented and the role of pulmonary lavage in management is illustrated. Six patients had associated diseases such as nocardiosis, leukemia, and silicosis. The broad spectrum of relationships found in alveolar proteinosis suggests that it represents one mechanism by which the lung responds to a variety of insults.
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PMID:Pulmonary alveolar proteinosis: relationship to silicosis and pulmonary infection. 736 Sep 77

Acute promyelocytic leukemia (APL) serves as a paradigm in clinical and biological leukemia research. Firstly, APL represents a model for the new therapeutic approach of differentiation therapy, taking advantage of the ability of APL cells to respond to retinoic acid treatment by terminal differentiation. Secondly, the 15;17 chromosomal translocation specific for APL leads at the molecular genetic level to a chimeric gene fusing the PML and RAR alpha genes and appears to be an instrumental, if not actually the causative event, in the neoplastic process. These unique characteristics of an otherwise rather rare disease have recently attracted intense research interest. As in other types of leukemia where continuous cell lines are powerful research tools, studies using APL-derived cell lines have contributed a large body of relevant data in efforts to unravel the pathobiology and leukemogenesis of APL. Three cell lines have been reported to be derived from patients with APL: HL-60, NB-4 and PL-21. Both HL-60 and PL-21 lack t(15;17) while NB-4 carries this cytogenetic hallmark pathognomonic for APL. Morphological and immunophenotypical examinations of the cell lines do not permit a clear assignment to any stage of myelomonocytic differentiation. Some additional data, such as expression of myeloperoxidase, monocyte-specific esterase and annexin VIII, together with the cytogenetic and molecular biological information, suggest that NB-4 is the only genuine promyelocytic leukemia cell line, whereas HL-60 may represent a discrete stage of differentiation between the late myeloblasts and the promyelocyte; PL-21 has distinct features associated with monocytic cells. These cell lines provide unique in vitro model systems for studying the cellular and molecular events involved in the proliferation and differentiation of normal and leukemic myelomonocytic cells.
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PMID:Leukemia cell lines: in vitro models for the study of acute promyelocytic leukemia. 750 Jun 43

Congenital leukemia is a rare disease in which a leukemic process is present at birth or immediately thereafter. The majority of cases presented in the literature were reported prior to the availability of contemporary immunophenotyping methods, and lineage assignment was often made on the basis of morphology alone. Congenital leukemias may be of various lineages, although, historically, monocytic and myelomonocytic congenital leukemias appear to be the most prevalent. We present two cases of congenital leukemia with detailed immunophenotypic and cytochemical characterization. One case is of the lymphoid lineage, and the second is of myelomonocytic lineage. Neither patient displayed trisomy 21.
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PMID:Congenital leukemia: report of two cases. 881 97

The ICD classification of the myelodysplastic syndromes (MDS), and the four main features of the descriptive epidemiology of the condition are discussed. MDS is: (1) a rare disease; which may be, (2) on the increase; (3) which primarily affects the elderly; and (4) which predominantly affects men. We reviewed four causal models pertaining to the relationship between MDS and leukemia. These models may be described as: (1) non-biological correlates; (2) biological correlates; (3) early phase; and (4) interactive cause. Each model is described and the evidence in support of or against each is presented.
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PMID:Epidemiologic perspectives on myelodysplastic syndromes and leukemia. 759 48

Transition of a (probably primary) cutaneous non-Hodgkin's lymphoma to T-stem cell leukaemia was observed in a case. Unusual clinical features, histological and laboratory data hampered the diagnosis-making of this rare disease. Using the ALL-BFM 90 HRG protocoll complete remission was achieved. Under the treatment varicella-zoster virus encephalitis took place.
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PMID:[Progression of cutaneous T cell lymphoma in leukemia]. 805 4

Rhinocerebral mucormycosis is a fulminating, devastating fungal disease, usually associated with debilitating diseases such as diabetes mellitus, leukaemia and immunosuppressive conditions. Ten patients with this rare disease have been treated over the past 14 years at the Beilinson Medical Centre. Nine patients had an underlying debilitating disease and one patient had latent diabetes mellitus which was diagnosed only after presentation of mucormycosis. Only two of the 10 patients survived. Early aggressive surgical debridement, together with amphotericin B and correction of underlying metabolic acidosis were found to be important factors associated with survival.
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PMID:Mucormycosis: experience with 10 patients. 854 76

Severe chronic neutropenia (SCN) include a heterogeneous group of diseases characterized by blood neutrophil counts chronically less than 0.5 x 10(9)/ L. In phase I-III studies in SCN patients, treatment with recombinant human granulocyte colony stimulating factor (r-metHuG-CSF; Filgrastim) resulted in a rise in the absolute neutrophil counts (ANC) to above 1.0 x 10(9)/L associated with a reduction in bacterial infections. Long-term treatment with filgrastim up to 8 years demonstrate a sustained ANC response, a significant reduction of the need for intravenous antibiotics and a dramatic improvement in the quality of life. In 1994 an international registry for severe chronic neutropenia (SCNIR) was established to improve care for chronic neutropenia and for further understanding the pathophysiology of this rare disease. Three-hundred and ten patients have been enrolled to this registry so far. Worldwide phase I-III studies with filgrastim and SCNIR provide information on 424 patients with severe chronic neutropenia. Adverse events include the development of acute myeloid leukemia in approximately 7% of the patients within the cohort of patients with congenital neutropenia (Kostmann's syndrome) suggesting that congenital neutropenia is a preleukemic syndrome. None of the patients with cyclic of idiopathic neutropenia developed leukemia suggesting that filgrastim is not involved in the development of leukemia.
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PMID:Pathophysiology and treatment of severe chronic neutropenia. 862 68

We report an infant born with a cutaneous nodular eruption and neutropenia. Skin biopsy specimens revealed an immature dermal infiltrate suggestive of leukemia cutis, but repeated peripheral blood and bone marrow examinations failed to demonstrate malignant cells. The eruption resolved spontaneously. At the age of 3 months, a second occurrence of maculopapular skin lesions led to discovery of an acute monoblastic leukemia with (9;11)(p21-22;q23) translocation. Congenital acute leukemia is a rare disease associated with skin infiltration in 25% to 30% of patients. Usually the diagnosis is easily made by peripheral blood examination and/or bone marrow aspirate. However, skin involvement may precede acute leukemia by several weeks. Although very rare, this event must be kept in mind.
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PMID:Congenital leukemia cutis preceding monoblastic leukemia by 3 months. 898 56

Idiopathic hypereosinophilic syndrome (HES) is a rare disease with no established effective therapy. It has been reported that interleukin-5 (IL-5) produced by helper T cells plays a major role in the proliferation of eosinophils. The nucleotide analogue 2-chlorodeoxyadenosine (2-CdA), which induces excellent clinical responses in hairy cell leukemia, is known to suppress helper T cells; therefore, we used 2-CdA, alone or in combination with cytarabine, to treat patients with idiopathic HES. 2-CdA alone and combined with cytarabine resulted in a rapid and sustained decrease in circulating eosinophils in two patients with idiopathic HES that was refractory to steroids, hydroxyurea and cytarabine. The efficacy of 2-CdA alone and combined with cytarabine exceeded by far that of cytarabine alone. However, reverse transcription-polymerase chain reaction (RT-PCR) did not show production of IL-5 or granulocyte-macrophage colony-stimulating factor mRNA in T cells as previously reported, and multiple cytokine receptors were found on eosinophils in idiopathic HES, suggesting that IL-5 may not be the sole cytokine involved in the regulation of idiopathic HES. The clinical efficacy of 2-CdA in idiopathic HES needs to be established on a large group of patients.
Leukemia 1997 Aug
PMID:2-Chlorodeoxyadenosine therapy for idiopathic hypereosinophilic syndrome. 926 99


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