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Volume 8, Issue 5 (Suppl) J Blood Disord Transfus, an open access journal
ISSN:2155-9864 Hematology 2017
November 08-09, 2017
November 08-09, 2017 | Las Vegas, USA
11th International Conference on
Hematology & Hematological Oncology
Catastrophic antiphospholipid syndrome and sarcoidosis: A case report
Nova Thomas John1, Madhavi Gorusu1, Katrina Collins2 and Joseph Di Giuseppe2
1Starling Physicians, USA
2Hartford Hospital, USA
C atastrophic antiphospholipid syndrome (CAPS) is a systemic autoimmune disease which occurs in <1% of patients with
Antiphospholipid syndrome (APS). It is the most severe variant of the classic APS, characterized by histopathologic and
clinical evidence of widespread small vessel microthrombi. The resulting inflammatory cytokine storm causes multi-organ
failure over a short period and laboratory confirmation of high antiphospholipid antibody titers. Sarcoidosis is a systemic
inflammatory disorder characterized by granulomatous inflammation of various organs. Although the association of APS and
sarcoidosis may be explained by shared immune dysregulation, cases with concurrent sarcoidosis and APS are extremely rare.
Here, we present the 12th reported case, presenting with digital gangrene and review the literature on CAPS. A 66 year-old
gentleman presented with rapidly progressive ischemic changes of extremities with skin ulcerations and gangrene of peripheral
digits. Autoantibodies testing revealed elevated levels of anti-beta2 glycoprotein IgM and anti-cardiolipin IgM antibodies.
Skin ulceration biopsy showed vasculitis with intravascular microthrombi deposition. First-line treatment was initiated for
“Probable CAPS” with anticoagulation, glucocorticoids and therapeutic plasma exchange. Subsequent, bone marrow biopsy
workup for acute leucopenia with lymphopenia, revealed non-necrotizing granuloma, suggestive of sarcoidosis. This was
further substantiated with high serum Angiotensin converting enzyme level. CAPS is a challenging systemic disease requiring
a high index of clinical awareness, as outcomes are poor without prompt recognition and early initiation of targeted multimodal
therapy. This case highlights the need for a collaborative team approach. It is also the first case reported of probable CAPS
associated with sarcoidosis of bone marrow.
Biography
Nova Thomas John has completed her MBBS degree from Kasturba Medical College, Manipal University, Karnataka, India and Internal Medicine Residency from
University of Illinois Urbana-Champaign, Illinois, USA. She is currently practicing as a Hospitalist Medicine Physician with Starling Physicians Group at Hartford
Hospital, Connecticut, USA since August 2015.
Nova Thomas John et al., J Blood Disord Transfus 2017, 8:5 (Suppl)
DOI: 10.4172/2155-9864-C1-028