Our purpose was to compile information on the haematological manifestations of
systemic lupus erythematosus (SLE), namely leucopenia,
lymphopenia,
thrombocytopenia, autoimmune haemolytic anaemia (AIHA),
thrombotic thrombocytopenic purpura (
TTP) and
myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation.
Neutropenia can lead to morbidity and mortality from increased susceptibility to
infection. Severe
neutropenia can be successfully treated with
granulocyte colony-stimulating factor. While related to disease activity, there is no specific
therapy for
lymphopenia. Severe
lymphopenia may require the use of prophylactic
therapy to prevent select
opportunistic infections. Isolated
idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation,
haemolytic anaemia, the
antiphospholipid syndrome and renal disease.
Thrombocytopenia can be regarded as an important prognostic
indicator of survival in patients with SLE. Medical, surgical and
biological treatment modalities are reviewed for this manifestation. First-line
therapy remains
glucocorticoids. Through our review, we conclude
glucocorticoids do produce a response in majority of patients initially, but sustained response to
therapy is unlikely.
Glucocorticoids are used as first-line
therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line
therapy.
Rituximab is promising in refractory and non-responding AIHA.
TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of
TTP and SLE, and a few patients with SLE have concurrent
TTP.
Myelofibrosis is an uncommon yet well-documented manifestation of SLE. We have compiled the cases that were reported in MEDLINE sources.