Paroxysmal nocturnal hemoglobinuria (PNH) is a type of intravascular hemolytic anemia caused by an acquired mutation in the PIG-A (phosphatidylinositol N acetylglucosaminyltransferase subunit A) gene in hematopoietic stem cells, leading to clonal proliferation of cells deficient in a functional component of the cell membrane called glycosyl phosphatidyl inositol (GPI). Classification into extravascular and intravascular hemolytic anemias is one such subdivision based on the site of hemolysis . They can be subdivided based on inheritance, structural cellular defects, point of hemolysis, and duration. Hemolytic anemia is a type of anemia caused by increased destruction of red blood cells (RBCs). The causes of anemias in our population are diverse and the workup of anemias can be very costly and cumbersome. He died two days later due to cardiorespiratory failure. On history taking, his family revealed non-compliance to treatment due affordability and availability issues. He failed to follow-up and was admitted 12 months later with an ischemic stroke and aspiration pneumonia. He was subsequently labelled as a case of PNH and was prescribed eculizumab and vitamin supplements along with monthly follow-ups. Qualitative assay for CD55 and CD59 was sent which came back negative, signifying the absence of these factors from the cell surface. Bone marrow aspirate and trephine biopsy revealed hypercellular marrow with mainly normoblastic and a few megaloblastic changes. He was investigated for autoimmune and hereditary causes of anemia by direct Coombs test, red cell antibody screen, quantitative glucose-6-phosphate dehydrogenase (G6PD) levels, and osmotic fragility test, all of which came back negative. Peripheral smear revealed anisocytosis, hypochromic microcytosis, macrocytosis, and pencil cells. His labs showed a hemoglobin (Hb) of 8g/dl, mean corpuscular volume (MCV) of 105fl and reticulocyte count of 4% with indirect hyperbilirubinemia (1.5 mg/dl). Splenomegaly could not be appreciated on examination but was visible on an abdominal ultrasound. On examination, he was vitally stable and the only clinically significant finding was pallor and jaundice. He had no significant past medical, family or drug history and had no history of weight loss, anorexia, intravenous drug abuse, or unprotected sexual intercourse. The patient had been worked-up for anemia at multiple hospitals over the preceding two years but had not been diagnosed. A 28-year-old Pakistani male presented to Hayatabad Medical Complex, Peshawar in August 2017, with weakness and generalized body aches for two years, dark urine for one year, jaundice for six weeks, and intermittent low-grade fever for three weeks. Informed consent was obtained from the patient and his family for this case report. We aim to present this case keeping in mind the rarity of this condition and the potentially fatal complications that can develop due to the lack of treatment. Diagnosing PNH might be a triumph for physicians but simultaneously is a curse for poor patients. The term zero-sum-game is used to describe a situation where one party gains at the expense of the other . Moreover, the availability of treatment is also a major issue in countries like Pakistan. Current treatment guidelines recommend the use of eculizumab to treat PNH but this treatment is quite expensive and is beyond the reach of most patients in developing countries. It can prove to be a diagnostic challenge for physicians due to its rarity and the numerous differentials that require ruling out. Moreover, despite thrombosis being a well-known complication of this condition, very few cases of arterial thrombosis have been documented in the literature. Although the literature contains a lot of information on PNH, the disease is rarely encountered in clinical practice. The disease has a slightly higher predisposition for females with 52.1% of diagnosed patients being women. The mean age at diagnosis is 50.0 years with a standard deviation of 18.6. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired form of intravascular hemolysis having a worldwide incidence of five to six cases per million and a prevalence of 16 cases per million.
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