80 years old lady was admitted via emergency department with increasing shortness of breath and cold fingers. she has no past medical history. Not on any medication
Evaluation in the emergency department revealed only acrocyanosis . Her saturation were unobtainable via pulse oxymetry.
System examinations were unremarkable
emergency blood test are as below
HB 43
MCV 127 (Significantly raised- normal range 82-95 )
WCC 12 , Neutrophils 7, Lymphocytes 5, CRP 100. Bilirubin 45, Creatinine 100
LDH 1200 Raised (Normal range 120-450)
CXR : NAD
Blood film examination showed roulaux formation
http://janaanhealth.org/wp-content/uploads/2019/01/CHAD-Film.pptx
Haematology team were contacted
Direct antiglobulin test IGM +4 CD3d positive
A diagnosis of Cold agglutinin disease were made
CHAD
Mainly idiopathic
underlying LPD or rarely viral /bacterial infection
usually refractory to Steroids unlike Warm autoimmune haemolytic anaemia
Management
look for underlying cause
cold avoidance
trial of prednisolone 1mg/kg
may need CD20 antibody Rituximab or treat in underlying LPD
Full antibody test at NHS BT
Virology
Folic acid life long 5mg po od
there are case report of success with Eculizumab and Valcade in refractory disease under clinical trial