POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder (PCD). The acronym, which was coined by Bardwick in 1980, refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal PCD, and skin changes. Continue reading
The International Myeloma Working Group (IMWG) consensus updates the disease definition of multiple myeloma to include validated biomarkers in addition to existing requirements of attributable CRAB features (hypercalcaemia, renal failure, anaemia, and bone lesions). Continue reading
The porphyrias are disorders of heme synthesis, which has eight steps. Each type of porphyria involves a defect, either inherited or acquired, in a pathway enzyme. When the defect is physiologically significant, it results in overproduction of pathway precursors preceding the defective step which enter the circulation and are excreted into urine or bile. The diseases have been grouped as acute hepatic porphyrias and photocutaneous porphyrias. The acute porphyrias are due to hepatic overproduction of the porphyrin precursors, delta aminolevulinic acid and porphobilinogen, and the symptoms are caused by injury primarily to the nervous system. Cutaneous porphyria is due to overproduction of photosensitizing porphyrins by the liver or bone marrow, depending on the type of porphyria.
Platelet transfusion may be indicated to prevent hemorrhage in patients with thrombocytopenia or platelet function defects.
The World Health Organization classification of lymphoid neoplasms updated in 2008 represents a worldwide consensus on the diagnosis of these tumors and is based on the recognition of distinct diseases, using a multidisciplinary approach.
The definition of plasma cell myeloma (PCM) has rested on identifying clinical and laboratory features that predict when a sufficient burden of plasma cells has accumulated that the patient will benefit from treatment.
The thalassemias are a group of inherited hematologic disorders caused by defects in the synthesis of one or more of the hemoglobin chains. Alpha thalassemia is caused by reduced or absent synthesis of alpha globin chains, and beta thalassemia is caused by reduced or absent synthesis of beta globin chains. Imbalances of globin chains cause hemolysis and impair erythropoiesis.
A peripheral-blood smear is a vital investigation tool in most cases to confirm a low platelet count and the presence or absence of other diagnostic features, such as red-cell fragmentation, platelet morphologic abnormalities, or evidence of dysplasia or hematinic deficiency.
Disseminated intravascular coagulation is a clinicopathological diagnosis of a disorder that is defined by the International Society on Thrombosis and Hemostasis (ISTH) as “an acquired syndrome characterized by the intravascular activation of coagulation with loss of localization arising from different causes”. This condition typically originates in the microvasculature and can cause damage of such severity that it leads to organ dysfunction. It can be identified on the basis of a scoring system developed by the ISTH.
The diagnosis of Iron Deficiency Anemia (IDA) requires that a patient be anemic and show laboratory evidence of iron deficiency. Red blood cells in IDA are usually described as being microcytic (i.e., mean corpuscular volume less than 80 um3 [80 fL]) and hypochromic, however the manifestation of iron deficiency occurs in several stages.