Hairy cell leukaemia (HCL) is a rare lymphoproliferative disorder associate
d with pancytopenia, splenomegaly and the presence of typical hairy B lymph
ocytes in the bone marrow and/or peripheral blood. The most significant com
plication relates to opportunistic infections that arise as a consequence o
f neutropenia and monocytopenia. HCL is occasionally associated with system
ic autoimmune disorders including polyarteritis nodosa and rheumatoid disea
se. Secondary autoimmune haemolytic anaemia (AIHA) appears to be rare. Mie
report on two cases of HCL complicated by fatal cold anti-i AIHA, Fulminant
haemolysis causing death is rare in cold AIHA and only a few individual ca
ses have been reported, none having anti-i specificity.