ENFERMEDAD DE WALDENSTROM PDF
Waldenstrom macroglobulinemia is a chronic, slow-growing lymphoproliferative disorder. It usually affects older adults and is primarily found in the bone marrow . Waldenström macroglobulinemia (WM) is a distinct clinicopathologic entity demonstrating lymphoplasmacytic lymphoma (LPL) in the bone. Waldenström macroglobulinemia is often an indolent disorder, and many patients are candidates for observation with careful monitoring.
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A pathologist identifies the particular lymphocytes that indicate WM.
In relation to the mechanisms involved in the pathophysiology of WM, the blocking of immunoglobulin isotype switching and the role of cytokines is noteworthy. Lymphoproliferative disorders X-linked lymphoproliferative disease Waaldenstrom lymphoproliferative syndrome Leukemoid reaction Diffuse infiltrative lymphocytosis syndrome.
In WM, medullary infiltrate consists of a monoclonal cellular population of small B-lymphocytes, in different maturation stages: A skeletal survey can help distinguish between WM and multiple myeloma.
Bone marrow tumour cells express the following antigen targets CD20 Enfermedzd monoclonal anti-CD20 antibody: Diagnosis and management of Waldenstrom’s macroglobulinemia. Typical Waldenstrom macroglobulinemia is derived from a B-cell arrested after cessation of somatic mutation but prior to isotype switch events. Immunophenotypic analysis of Waldenstrom’s macroglobulinemia. Should treatment be started it should address both the paraprotein level and the lymphocytic B-cells.
Its etiology is unknown, but several studies suggest a possible causal relationship with autoimmune diseases, exposure to environmental factors and chronic antigenic stimulation, such as infection with the hepatitis C virus HCV.
Waldenström’s macroglobulinemia – a review
The main clinical manifestations associated with the hyperviscosity syndrome are bleeding enfermeddad, bleeding gums and gastrointestinal bleedingocular changes papilledema, blindness, blurred vision and retinal changes: Four brothers with Waldenstrom’s macroglobulinemia. Nature Clinical Practice Neurology.
Holland-Frei Enferjedad Medicine 6th ed. In patients with peripheral neuropathy, such as progressive symmetrical numbness of the limbs, burning sensation and fe, pain in the feet and hands. Views Read Edit View history. From Wikipedia, the free encyclopedia. Rare cancers Hematologic malignant neoplasms Vascular-related cutaneous conditions Lymphoma.
Older diagnosis and treatments resulted in published reports of median survival of approximately 5 years from time of diagnosis. Assess the bone marrow infiltration by lymphocytes, the infiltration pattern and cell morphology. Immunophenotypic variations may occur. Peripheral neuropathy in essential mixed cryoglobulinemia.
WM occurs more frequently in older adults. The light chain of the monoclonal protein is usually the kappa light chain. Chemistry tests include lactate dehydrogenase LDH levels, uric acid levels, erythrocyte sedimentation rate ESRkidney and liver function, total protein levels, and an albumin-to-globulin ratio.
LPLs are rare and indolent cancers of mature B-lymphocytes, which predominantly involve the bone marrow and, less commonly, the spleen, lymph nodes, peripheral blood and other organs.
Monoclonal IgM may exhibit “cold agglutinin” activity, binding to erythrocyte antigens at a temperature lower than physiological temperature, determining the development of chronic cold antibody hemolytic anemia. Rituximab is an IgG1 anti-CD20 monoclonal antibody. Soluble CD27 is a faithful marker of disease burden and is unaffected by the rituximab-induced IgM flare, as well as by plasmapheresis, in patients with Waldenstrom’s macroglobulinemia.
Detection of monoclonal gammopathy – homogeneous peak, high, narrow base, usually in the area of gamma globulins. J Dtsch Dermatol Ges. International Journal of Laboratory Hematology. Arch Pathol Lab Med.
Immunophenotyping should be interpreted simultaneously, verifying its consistency with the results of a bone marrow biopsy. The article refers to them as “adverse covariates”.
June 04, ; Accepted: Fadil A, Taylor DE.
WM patients are at higher risk of developing second cancers than the general population, but it is not yet clear whether treatments are contributory. Other immunophenotypic characteristics of neoplastic B-lymphocytes: The symptoms of malabsorption, diarrhea, obstipation or bleeding may indicate involvement of the gastrointestinal tract.
Treatment of patients with asymptomatic WM does not improve the quality of life of patients, or increase their survival, being recommended, therefore, their follow-up. Transplantation of hematopoietic stem cells is indicated in younger patients with multiple recurrences or who have been refractory to previous treatments.
It is also responsible for the development of distal symmetrical sensorimotor polyneuropathy or multiple mononeuropathy with axonal degeneration. Some of these markers are particularly useful for studying the bone marrow infiltrate in the bone biopsy through immunohistochemical and other studies for the immunophenotypic characterization of B-lymphocytes by flow cytometry. Additional tests such as computed tomography CT or CAT scan may be used to evaluate the chest, abdomen, and pelvis, particularly swelling of the lymph nodes, liver, and spleen.
ETIOLOGY Its etiology is unknown, but several studies suggest a possible causal relationship with autoimmune diseases, exposure to environmental factors and chronic antigenic stimulation, such as infection with the hepatitis C virus HCV.
Recently, autologous bone marrow transplantation has been added to the available treatment options. Another study showed the possibility of isotype switching occurring in vivo.
The most frequent neurological disorder is a demyelinating distal symmetrical sensorimotor peripheral neuropathy, which manifests itself slowly and progressively, causing paresthesia and asthenia. Nodular, interstitial, diffuse or mix of all three. Although isotype switching is rarely seen in WM, according to some studies it is possible that it occurs wladenstrom vivo and in vivo.