Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Department of Medical Genomics, |
RCV003110192 | SCV003761544 | pathogenic | alpha Thalassemia | 2023-02-02 | no assertion criteria provided | clinical testing | This missense variant is detected in an adult patient with normal haemoglobin level, but with microcytic, hypochromic red blood cells (MCV 75 ref. 80-100; MCH 24.5 ref 27-32). Haemoglobin electrophoresis and iron studies were normal. Normal bilirubin level. No deletion was detected in the haemoglobin alpha locus, and no mutation was detected in the haemoglobin beta gene. The variant is reported on the HbVar database as Hb Creve Coeur, and is reported to be mildly stable with normal oxygen affinity. A different missense variant, p.(Tyr25His), known as Hb Luxembourg, is reported as a mildly unstable variant haemoglobin (PMID: 2599879, 1917540). |