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Conceptual organization of hematologic malignancies Organization of tumors of the hematopoietic and lymphoid tissues as described by the World Health Classification 2008. Swerdlow, SH, Campo, E, Harris, NL, et al. (Eds). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon 2008.   Add caption

Essentials of Diagnosis X-linked recessive disorder seen commonly in American black men. Episodic hemolysis in response to oxidant drugs or infection. Minimally abnormal peripheral blood smear. Reduced levels of glucose-6-phosphate dehydrogenase between hemolytic episodes. General Considerations Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a hereditary enzyme defect that causes episodic hemolytic anemia because of the decreased ability of red blood cells to deal with oxidative stresses. Oxidized hemoglobin denatures and forms precipitants called Heinz bodies. These Heinz bodies cause membrane damage, which leads to removal of these cells by the spleen. Numerous types of G6PD enzymes have been described. The normal type found in whites is designated G6PD-B. Most American blacks have G6PD-A, which is normal in function. Ten to 15 percent of American blacks have the variant G6PD designated A–, in which there is only 15% of normal enzyme activity, and enzyme activity declines ...

Paroxysmal nocturnal hemoglobinuria is an acquired clonal stem cell disorder that results in abnormal sensitivity of the red blood cell membrane to lysis by complement. The underlying cause is a defect in the gene for phosphatidylinositol class A (PIC-A), which results in a deficiency of the glycosylphosphatidylinositol (GPI) anchor for cellular membrane proteins. In particular, the complement-regulating proteins CD55 and CD59 are deficient. Paroxysmal nocturnal hemoglobinuria should be suspected in confusing cases of hemolytic anemia or pancytopenia. The best screening test is flow cytometry to demonstrate deficiency of CD59 on red blood cells. This test has largely replaced the classic sucrose hemolysis test. Clinical Findings SYMPTOMS AND SIGNS Classically, patients report episodic hemoglobinuria resulting in reddish brown urine. Hemoglobinuria is most often noticed in the first morning urine, probably because of its increased concentration. In addition to being prone to anemia, th...

Adult acquired pure red cell aplasia is rare. It appears to be an autoimmune disease mediated either by T lymphocytes or (rarely) by an IgG antibody against erythroid precursors. In adults, the disease is usually idiopathic. However, cases have been seen in association with systemic lupus erythematosus, chronic lymphocytic leukemia, lymphomas, or thymoma. Some drugs (phenytoin, chloramphenicol) may cause red cell aplasia. Transient episodes of red cell aplasia are probably common in response to viral infections, especially parvovirus infections. However, these acute episodes will go unrecognized unless the patient has a chronic hemolytic disorder, in which case the hematocrit may fall precipitously. The only signs are those of anemia unless the patient has an associated autoimmune or lymphoproliferative disorder. The anemia is often severe and normochromic, with low or absent reticulocytes. Red blood cell morphology is normal, and the myeloid and platelet lines are unaffected. The bon...