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Agranulocytosis is a rare but potentially life-threatening disorder of the blood, characterized by an almost complete absence of granulocytes in the blood. Granulocytes are a critical subtype of white blood cells (leukocytes) that play a central role in the immune defense, particularly in the fight against bacterial infections. Without granulocytes, the body becomes highly susceptible to  immune defense, , and even minor bacterial invasions can lead to severe, rapidly progressing complications. Definition and Clinical Criteria Agranulocytosis is defined as a severe reduction in the number of granulocytes, typically neutrophils, in the peripheral blood. Medically, this condition is diagnosed when the absolute neutrophil count (ANC) falls below 500 cells per microliter of blood . At this level, the immune defense is critically impaired, exposing patients to a high risk of overwhelming infections. Metamizole by other names:  Generic names  Dipyrone Sulpyrine Noramidopyr...

Hematology March 2021: SWA Personal image    Introduction Acute Intermittent Porphyria (AIP) is a rare, inherited metabolic disorder that affects the body’s ability to produce heme , a vital component of hemoglobin. The disease is caused by a partial deficiency of the enzyme porphobilinogen deaminase (PBGD) , leading to the accumulation of toxic heme precursors— aminolevulinic acid (ALA) and porphobilinogen (PBG) —in the body. These compounds can cause acute neurovisceral attacks , particularly under conditions of physiological stress. AIP does not typically cause petechiae , which are small pinpoint red or purple spots on the skin caused by bleeding under the surface. However, understanding the full clinical picture—including the role of medications like warfarin , treatments like hematin , and the potential influence of viral infections —is essential for managing and preventing complications in patients with AIP. 1. Genetic Cause and Pathophysiology of AIP Gene Involv...