Investigation of persistent hypochromic microcytosis unmasks hemoglobin Evanston [alpha 14 (A12) Try--> Arg] in a patient of cyclic thrombocytopenia preceding Takayasu's disease.
We report an unusual north Indian patient with Hemoglobin Evanston [alpha 14 (A12) Try --> Arg] who had acquired cyclic thrombocytopenia (10-1230 x 10(9)/l periodic oscillation of four week duration) which recovered without any specific therapy. She later developed Takayasu's disease and underwent three corrective stents. She is presently in clinical remission and is on regular follow up. To the best of our knowledge our patient is the first report of Hb Evanston from the indigenous population of India and highlights the need to look for point mutations in the alpha globin gene, which may interact with thalassemia or other hemoglobinopathies, in atypical cases. The association of these three disorders in our patient is possibly unrelated though an immune basis for the cyclic thrombocytopenia and Takayasu's disease is likely as seen in this report.
Das
Reena
R
Department of Haematology, PGIMER, Chandigarh, India.
Garewal
Gurjeewan
G
Shaji
R V
RV
Ahluwalia
Jasmina
J
Bali
H K
HK
Varma
S
S
D000328:Adult; D000747:Anemia, Hypochromic / Q000150:complications; D005260:Female; D006455:Hemoglobins, Abnormal / Q000235:genetics; D006801:Humans; D017354:Point Mutation; D015607:Stents; D013625:Takayasu Arteritis / Q000209:etiology / Q000601:surgery*; D013921:Thrombocytopenia / Q000150:complications
D006455:Hemoglobins, Abnormal; C039216:hemoglobin Evanston