"In vivo" and "in vitro" demonstration of hemoglobin C crystals in non-splenectomized patients.
We studied 12 Hb C carriers: 4 homozygotic Hb CC and 8 heterozygotic. We observed the presence of free crystals in the peripheral blood of the homozygotes but in none of the heterozygotes. However, after incubation with 3% NaCl we were able to detect crystals in the heterozygotes (Hb AC and Hb SC), and in the homozygotes (Hb CC). In patient 04 (P04) less crystals formation occurred due to inhibition of the process by the presence of elevated levels of Hb F (12.2%). All the homozygotic patients had a splenomegaly of 3 to 6 fingerbreadths. We believe that the spleen wears off with time, thus allowing the passage of crystals to the peripheral blood. This finding might be associated with splenic insufficiency without a reduction of its dimensions. Finally, the finding of crystals in the peripheral blood permitted the diagnosis of Hb C obviating the need for electrophoresis.
Araújo
J T
JT
Laboratório de Diagnóstico e Investigação das Hemoglobinopatias e Talassemias do Instituto de Medicina Tropical de São Paulo, HCFMUSP, São Paulo, SP, Brasil.
Batissoco
A C
AC
Bodemeier
L
L
D003460:Crystallization; D004587:Electrophoresis, Agar Gel; D006444:Hemoglobin C / Q000737:chemistry*; D006445:Hemoglobin C Disease / Q000175:diagnosis*; D006720:Homozygote; D006801:Humans; D013154:Spleen / Q000503:physiopathology; D013163:Splenomegaly / Q000097:blood*
D006444:Hemoglobin C