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2015 ; 2
(1
): e000078
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Haematological manifestations of lupus
#MMPMID25861458
Fayyaz A
; Igoe A
; Kurien BT
; Danda D
; James JA
; Stafford HA
; Scofield RH
Lupus Sci Med
2015[]; 2
(1
): e000078
PMID25861458
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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.
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