Inherited haemoglobin disorders are endemic through Africa, South-East Asia and the Caribbean and the Mediterranean, where the high allelic frequency offers some protection against infectious disease, particularly malaria. They are conventionally divided into disorders of haemoglobin structure (e.g. sickle cell) and haemoglobin production (e.g. the thalassaemias).
By their nature, inherited haemoglobin disorders will always be acquired the recipient of a haematopoietic stem cell transplant, assuming engraftment occurs. However, many carrier states and compound heterozygotes have a clinical silent disease which may be well-tolerated by the recipient. In addition, for ethnic minority recipients who may have an extremely limited choice of donors, the risk of an acquired haemoglobinopathy may be outweighed by the chance of cure.
Must not donate if: mother or father homozygous or heterozygous for inherited haemoglobin disorders and infant affected.
Discretionary: If the cord blood or infant/child is tested for the condition and the infant is shown to be unaffected or heterozygous (trait), accept and inform the transplant centre.
QUALIFIED, see below
QUALIFIED, see below
Each donor with a haemoglobinopathy should be assessed by a physician on a case-by-case basis. In general:
Thalassaemia major or intermedia
Sickle cell disease (HbSS, HbSC, HbSBthal, HbSD)
High affinity haemoglobin
Other clinically significant structural or functional haemoglobinopathies.
Alpha or beta thalassaemia trait
Sickle cell trait
HbC, HbDPunjab/Oman, HbE traits
Other asymptomatic traits or compound heterozygotic haemoglobinopathies e.g. HbC/a-thal trait.
There is no evidence of clinically significant sickling during mobilized PBSC collection in those with sickle cell trait. However, clumping of sickle cells has been observed during post-collection processing. In these circumstances, it is advisable that donors with sickle cell trait should donate by BM.
Kang EM, Areman EM, David-Ocampo V, Fitzhugh C, Link ME, Read EJ et al. Mobilization, collection, and processing of peripheral blood stem cells in individuals with sickle cell trait. Blood 2002; 99(3): 850-5.
Castro O, Hardy KP, Winter WP, Hornblower M, Meryman HT. Freeze preservation of sickle erythrocytes. Am J Hematol 1981; 10(3): 297-304.
Meryman HT, Hornblower M. Freezing and deglycerolizing sickle-trait red blood cells. Transfusion 1976; 16(6): 627-32.
Adler BK, Salzman DE, Carabasi MH, Vaughan WP, Reddy VV, Prchal JT. Fatal sickle cell crisis after granulocyte colony-stimulating factor administration. Blood 2001;97(10):3313-4.