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)


Blood film examination showed roulaux formation


Haematology team were contacted

Direct antiglobulin test IGM +4 CD3d positive

A diagnosis of Cold agglutinin disease were made



Mainly idiopathic

underlying LPD or rarely viral /bacterial infection

usually refractory to Steroids unlike Warm autoimmune haemolytic anaemia


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


Folic acid life long 5mg po od

there are case report of success with Eculizumab and Valcade in refractory disease under clinical trial