Chronic urticaria associated with chronic myelomonocytic leukemia.

Although chronic urticaria is usually idiopathic, in rare cases it may be a sign of underlying malignancy. We describe the first case of chronic urticaria associated with chronic myelomonocytic leukemia. The urticarial lesions healed successfully with etoposide, an antineoplastic agent. This case demonstrates that cases of chronic urticaria should not to be labeled as idiopathic until diligent evaluation has failed to reveal a cause.     

Chronic neutrophilic leukemia and chronic myelomonocytic leukemia: WHO defined

The World Health Organization (WHO) classification of myeloid disorders has provided updated parameters for the consistent diagnosis of two previously less than optimally defined chronic myeloid disorders, CNL and CMML. The classification of these disorders, which had been controversial, is now better defined and provides more clinically and biologically relevant disease definitions to enable uniform diagnosis and a framework to evaluate natural history and therapeutic interventions. CNL is now recognized as a distinct entity among the chronic myeloproliferative disorders and CMML is included within the new category of 'myelodysplastic/myeloproliferative diseases' (MDS/MPD). Predominant neutrophilia defines CNL whereas CMML is defined by predominant and monocytosis. In each case these defining features must be distinguished from reactive causes for the same in the absence of clear evidence of myeloid clonality (CNL and CMML) or dysplasia (CMML). The exclusion of underlying bcr/abl-driven oncogenesis is an essential component in the diagnosis of these chronic leukemic processes. The optimal therapy for both CNL and CMML remains uncertain. Current management decisions are based on small studies or extrapolated from therapeutic strategies that are effective in similar chronic, clonal myeloid disorders. Given the potential for evolution to acute leukemia or progressive refractory leucocytosis or cytopenias, allogeneic stem cell transplantation might be appropriate for younger patients. Continued reporting and investigation of specific therapeutic strategies and responses must be encouraged.     

Chronic lymphocytic leukemia.

Significant strides have been made in our understanding of the biology and treatment of B cell chronic lymphocytic leukemia. Recent studies have defined cytogenetic and molecular lesions that may be responsible for leukemogenesis or disease progression. Molecular analyses of immunoglobulin genes have delineated two or more subgroups of chronic lymphocytic leukemia that may differ in their clinical behavior. Research in the biochemistry of chronic lymphocytic leukemia has provided insight into the noted resistance of leukemia cells to cytotoxic drugs. Investigations into the immunology has revealed mechanisms whereby chronic lymphocytic leukemia cells can contribute to the immune deficiency that commonly develops in patients with this disease. Clinical studies have delineated factors that are helpful in predicting prognosis and have provided data on promising new therapies for patients with this disease, including stem cell transplantation, monoclonal antibodies, and gene therapy.     

Chronic myelomonocytic leukemia in childhood

A four-year-old child with recurrent infections and increasing hepatosplenomegaly over a three-year period was evaluated. Increased numbers of myeloid precursors packed the bone marrow and infiltrated the peripheral blood. A diagnosis of chronic myelogenous leukemia (CML) was considered but could not be confirmed by laboratory studies appropriate for the types of CML usually observed in childhood. Examination of the patient's peripheral blood smears revealed many atypical monocytoid cells with unipolar hairy projections. Scanning electron microscopy showed these to be leukemic monoblasts with characteristic broad-based ruffles on the cell surface. A population of myeloid precursors possessing narrow ridge-like profiles was also observed. Progressive infiltration of the spleen caused hypersplenism which necessitated splenectomy. Subsequently, massive liver and bone marrow involvement led to the patient's death. Terminally, the proliferating blast cells were demonstrated to be leukemic monoblasts by analysis of cytochemical staining patterns, surface immunoglobulins, serum lysozyme levels, and monocyte-mediated antibody-dependent cellular cytotoxicity studies. The findings in this case are most compatible with a diagnosis of chronic myelomonocytic leukemia (CMML), a condition not previously described in childhood. Several myeloproliferative disorders with prolonged survival have been reported in children, but special studies were not performed to determine which cell lines were abnormally proliferating. The similarities between these children and our patient with CMML suggest that monocyte studies may be useful in the diagnosis of these unusual disorders, provide insights into their pathogenesis, and aid in the selection of appropriate therapy.     

Chronic lymphocytic leukemia

Chronic lymphocytic leukemia continues to attract much basic and clinical research interest. Despite recent advances, the disease still has no established cure. Nonetheless, significant strides have been made in our understanding of the genetics, biology, and clinical staging of this disease. This understanding may improve our ability to segregate patients into subtypes that differ in their cytogenesis, propensity toward disease progression, or response to standard or innovative forms of therapy. Finally, several promising new modalities of treatment are being evaluated in clinical trials, involving novel drugs or drug-combinations, monoclonal antibodies, stem cell transplantation, or gene therapy.     

Chronic lymphocytic leukemia

Chronic lymphocytic leukemia is a neoplasm characterized by the accumulation of monoclonal lymphocytes in blood, bone marrow, and other tissues. Staging of the disease is based on lymphoid mass and the presence of anemia or thrombocytopenia, with prognosis also related to the pattern of bone-marrow involvement, chromosomal abnormalities, and surface membrane phenotype. Transformation to a diffuse large-cell lymphoma or to prolymphocytic leukemia may occur as a terminal event. A number of biochemical and surface membrane abnormalities that may suggest approaches for future clinical investigations have been described.     

Chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in the western world and is due to the accumulation of mature B lymphocytes in the peripheral blood, bone marrow and secondary lymphoid organs. The leukemic cells show a distinct phenotype, which is essential to reach the correct diagnosis. Despite the phenotypic homogeneity, the clinical outcome may be significantly different. Some patients have an indolent leukemia, with long survival while others experience an aggressive disease, with early and frequent need of treatment. At present, no chemotherapeutic regimens can be considered curative and all patients will die with (or because of) their disease. In recent years, research on CLL has led to important discoveries that help defining patients' prognosis at the moment of diagnosis. These prognostic factors, which are derived from the biological features of the leukemic lymphocytes, are now rapidly moved into the clinical arena. They are used to stratify patients in selected clinical trials to assess the value of early and more modern treatments, which are becoming available to hematologists.     

Amyloidosis associated with chronic lymphocytic leukemia.

Chronic lymphocytic leukemia (CLL), the most common form of leukemia in Western countries, rarely induces glomerular disease, but membranoproliferative glomerulonephritis or immunotactoid glomerulopathy has been reported. The proliferating cells in CLL are of mature B-cell origin and produce monoclonal immunoglobulin (Ig), thus leading to various kinds of autoimmune disorders or immunotactoid glomerulopathy. Although there have been a few reported cases of amyloidosis accompanying CLL, the type of amyloid fibrils has not been demonstrated nor described in detail, particularly regarding monoclonal Ig productivity. We report a rare case of amyloidosis associated with CLL, in which we detected ?-light chain type monoclonal Ig in the sera, urine, and on the surface membrane of lymphocytes, and discuss an association between monoclonal Ig-related disease and non-Hodgkin's lymphoma.     

Chronic lymphocytic leukemia associated with von Recklinghausen neurofibromatosis.

We report a 62-year-old female, with von Recklinghausen neurofibromatosis and chronic lymphocytic leukemia, whose mother and son both had neurofibromatosis and died of digestive tract cancers. The patient died of pneumonia 3 years after the initiation of therapy. Leukemia reported in association with neurofibromatosis are predominantly nonlymphocytic and limited to childhood. The type of association found in our patient has not been reported previously.