[HbH disease--a rare differential diagnosis in a patient with anemia and abdominal pain].

Abstract

CASE REPORT:

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.

CONCLUSION:

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.

• Authors: Ursula Fabry, Elisabeth Kohne, Oliver Galm, Rainhardt Osieka
• Journal: Medizinische Klinik (Munich, Germany : 1983) (Med Klin (Munich)) Vol. 98 Issue 6 Pg. 335-8 (Jun 15 2003) ISSN: 0723-5003 [Print] Germany
• Vernacular Title: HbH-Krankheit--eine seltene Differentialdiagnose bei Anämie und Oberbauchschmerzen.
• PMID: 12811418 (Publication Type: Case Reports, Comparative Study, English Abstract, Journal Article)

Chemical References

• Hemoglobins
• hemoglobin B

Topics

• Abdominal Pain (etiology)
• Anemia, Hemolytic (diagnosis)
• Anemia, Hypochromic (diagnosis)
• Chromatography, High Pressure Liquid
• Diagnosis, Differential
• Electrophoresis
• Genotype
• Hemoglobins (analysis)
• Humans
• Male
• Middle Aged
• Mutation
• Prognosis
• alpha-Thalassemia (complications, diagnosis, genetics)