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2017 ; 2017
(ä): 5797041
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gab.com Text
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English Wikipedia
Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient-A High
Suspicion Is Still Needed
#MMPMID28620416
Ibrahim U
; Kedia S
; Garcia G
; Atallah JP
Case Rep Med
2017[]; 2017
(ä): 5797041
PMID28620416
show ga
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and
laboratory features of vascular thrombosis, pregnancy loss, and persistent
antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the
activation of endothelial cells, monocytes, platelets, and complement by aPLs.
Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present
a case of a 34-year-old male referred for pancytopenia and splenomegaly. On
examination, he had decreased sensation and 4/5 power in the left upper
extremity. A lacy, purplish rash was noted on the trunk and upper extremity. MRI
of brain showed acute/subacute lacunar infarctions. Laboratory studies revealed
an elevated lactate dehydrogenase level, bilirubin and ferritin, decreased
haptoglobin, and positive Coombs test. Antinuclear antibody test was negative and
antiphospholipid antibody panel revealed positivity for anti-cardiolipin IgG and
IgM, antiphosphatidylserine IgG, and anti-?2-glycoprotein IgG. The patient was
diagnosed with primary APS. Pancytopenia is relatively rare in primary APS and is
more often seen in secondary APS. Our patient demonstrated involvement of
multiple organ systems as well as livedo reticularis and autoimmune-related
findings such as Raynaud phenomenon and Coombs positive hemolytic anemia. We
discuss the various clinical and laboratory findings in patients with APS that
aid in diagnosis, as well as important management considerations.