الفهرس 
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Abstract
Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that affects multiple organ systems including the skin, kidneys, blood and brain. The exact cause is unknown but genetic, hormonal and environmental factors may all be involved in its development. It is one of a small number of truly multisystem disorders .The heterogeneous nature of the disease can result in delayed diagnosis.
There is no diagnostic test specific for SLE and as such the diagnosis remains a clinical one, relying on a combination of clinical and laboratory features. People with lupus often have features that are not specific to lupus , these include fever, fatigue, weight loss, and hair loss, heartburn, and poor circulation to the fingers and toes.
SLE can present with hematological manifestations alone or along with musculoskeletal, skin or other system involvement. In cases with hematological abnormalities as the predominant or only manifestations the diagnosis may be delayed or missed at the time of presentation. SLE is more a hematological disorder rather than a Rheumatologic disorder. A significant number do not satisfy the American College of Rheumatology (ACR) criteria at the time of diagnosis but do so on follow up.
The major hematologic manifestations of SLE are anemia, leukopenia, thrombocytopenia, and the anti-phospholipid antibody syndrome. The hematological changes, though very commonly seen, are not properly evaluated or estimated and are not given enough representation. It is only natural to expect hematological manifestations more often than others, since blood and blood vessels together contain more diverse number of antigens than any other organ in the body and in SLE auto antibodies are known to develop against any antigen or tissue.
The most common cause of anemia in SLE is anemia of chronic disease, which is result of prolonged inflammation leading to the production of hepcidin by
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the liver. Hepcidin up-regulates ferroportin causing the sequestration of iron in the bone marrow which leads to impaired erythropoiesis.
Leukopenia is a typical feature of SLE which may occur as a result of lymphopenia, neutropenia or a combination of both. Lymphopenia per se can predispose to autoimmunity and can also be a consequence of disease activity in the setting of active SLE .The prevalence of lymphopenia in SLE ranges from 20 to 81%.
Thrombocytopenia is a well-recognized hematological manifestation of SLE and its prevalence in the published literature ranges from 7%–30%. It constitutes one of the hematological classification criteria for SLE .Moreover, thrombocytopenia in SLE can be a complication of bone marrow suppression associated with immunosuppressive therapy such as azathioprine.
Hypercoagulability in SLE and thrombosis is due to multiple hits to the clotting system (the multiple-hit theory). It has been shown that elevations of procoagulant factors in combination have an additive effect on thrombosis. In lupus, this includes lupus-specific and non- specific thrombogenic factors.
Enlargement of lymph nodes occurs in approximately 50% of patients with SLE. It is more frequently noted at disease onset or during exacerbations.
Splenomegaly occurs in 10%-46% of SLE patients, particularly during active disease.