Status:
Ready to upload
Record number:
1205
Adverse Occurrence type:
MPHO Type:
Estimated frequency:
Rare
Time to detection:
11 days
Alerting signals, symptoms, evidence of occurrence:
Patient with hypochromic, microcytic anemia (Hb 7.1 g/dL, MCV 79, MCH 25.9), transfused with two units of RBC but failed to elicit the expected increase in Hb. Over the next 7 days received 7 units with no increase in Hb but increased LDH, total bilirubin and decreasing haptoglobin. On day 11 noted to be jaundiced with bilirubin of 17.9 mg/dL, elevated reticulocytes and positive direct antiglobulin test with spherocytes.
Demonstration of imputability or root cause:
In a two stage indirect antiglobulin test, the patient’s plasma caused hemolysis of LKE-S cells but not p, Pk, or LKE-N cells. Transfusion of P+ RBCs compatible by a prewarmed technique had shortened RBC survival with laboratory evidence of hemolysis. On HPTLC immunostaining, the patient’s plasma strongly bound monosialo-galactosyl- globoside (MSSG) with weak binding to galactosylgloboside (Gb5). This is the first example of a clinically significant anti-LKE in the setting of a rare weak P background.
Imputability grade:
3 Definite/Certain/Proven
Groups audience:
Keywords:
Suggest new keywords:
LKE, indirect antiglobulin, MSSG, Gb5,
Reference attachment:
Suggest references:
Cooling. L., Dake, L.R., Haverty, D., Mullis, N., Ellis, S., Shayman, J. and Judd, W.J. (2015). A hemolytic anti-LKE associated with a rare LKE-negative, "weak P" red blood cell phenotype: alloanti-LKE and alloanti-P recognize galactosylgloboside and monosialogalactosylgloboside (LKE) antigens. Transfusion 55(1):115-28.
Expert comments for publication:
LKE is related to the P antigens and is a high incidence antigen. Anti-P and -PP1Pk are naturally occurring antibodies invariably present in individuals with the very rare Pk and p phenotypes.