Abstract

MONDRAGON-CARDONA, Álvaro; ROJAS-MIRQUEZ, Johana; JIMENEZ-CANIZALES, Carlos Eduardo  and  UMANA-GIRALDO, Héctor Jairo. Paroxysmal Nocturnal Hemoglobinuria: case report. Investig. andina [online]. 2013, vol.15, n.26, pp.716-723. ISSN 0124-8146.

The Paroxysmal Nocturnal Hemoglobinuria is an infrequent cause of pancytopenia; although it has an approximate incidence of1/100.000people, it always should to be taken into account due the possible consequences in a patient's health. PNH could be fatal in some patients. This case is a 26 years old man that has been having for the last three years on and off symptoms like dyspnea, fever, asthenia, abdominal pain, gingival hemorrhage, epistaxis, hypercolored urine intermittently. In the physical exam we have a pale patient, slightly jaundiced and with petechiae. The patient has been going to the doctor's office for the last three years with similar concerns, accompanied with infectious episodes. The last episode, the patient presented with an hemoglobin of 4,7g/dL, a hematocrit of 14%, leucocytes of 200 x mm3, platelets of 1000 x mm3, reticulocytes of 4%, and a negative Coombs test. The principal cause of pancytopenia in PNH is the sensitivity of the blood cells to complement that cause cell lysis. This pathology has different forms of presentation: One of the forms presents with signs of hemolysis and bone marrow failure. This pathology has a variable course with constant exacerbations and remissions, or causing death little time after the diagnosis depending on the number of affected cells. The diagnosis is made with the Ham test, the saccharose test or flow cytometry. Because of the history of frequent infections, hemorrhages, petechiae and the laboratory results, he is taken as a patient with a possible bone marrow failure. Recommendations are made for his possible diagnosis of PNH and a Ham test is ordered.

Keywords : Anemia; Pancytopenia; Paroxysmal Nocturnal Hemoglobinuria.

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