[HbH disease--a rare differential diagnosis in a patient with anemia and abdominal pain].
METHODS
A 45-year-old Vietnamese male was admitted to hospital with severe hypochromic anemia and acute abdominal pain. The peripheral blood smear showed extreme anisocytosis and poikilocytosis as well as teardrops and target cells. Hemoglobin electrophoresis and brilliant cresyl blue staining revealed hemoglobin H (HbH) disease with an infection-associated hemolytic crisis.


CONCLUSIONS
In the diagnostic workup of hemolytic and hypochromic anemia, HbH disease as a special type of alpha-thalassemia should be considered early. In patients from Eastern Asia with a mean corpuscular hemoglobin (MCH) < 25 pg, hemoglobin analysis should be performed in order to avoid unnecessary diagnostic procedures. The prognosis of HbH disease is generally favorable, and symptomatic treatment is only recommended during hemolytic crises in association with, e.g., infections or pregnancy.
Fabry
Ursula
U
Medizinische Klinik IV, Universitätsklinikum Aachen. ufabry@ukaachen.de
Kohne
Elisabeth
E
Galm
Oliver
O
Osieka
Rainhardt
R
D015746:Abdominal Pain / Q000209:etiology*; D000743:Anemia, Hemolytic / Q000175:diagnosis*; D000747:Anemia, Hypochromic / Q000175:diagnosis*; D002851:Chromatography, High Pressure Liquid; D003937:Diagnosis, Differential; D004586:Electrophoresis; D005838:Genotype; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011379:Prognosis; D017085:alpha-Thalassemia / Q000150:complications / Q000175:diagnosis* / Q000235:genetics
D006454:Hemoglobins; C020622:hemoglobin B