Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed
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 compleme...
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Wiley
2017-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2017/5797041 |
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| author | Uroosa Ibrahim Shiksha Kedia Gwenalyn Garcia Jean Paul Atallah |
| author_facet | Uroosa Ibrahim Shiksha Kedia Gwenalyn Garcia Jean Paul Atallah |
| author_sort | Uroosa Ibrahim |
| collection | DOAJ |
| description | 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-β2-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. |
| format | Article |
| id | doaj-art-ca1ef6ccb92847c09b1e6e955a493ffd |
| institution | OA Journals |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
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| series | Case Reports in Medicine |
| spelling | doaj-art-ca1ef6ccb92847c09b1e6e955a493ffd2025-08-20T02:19:37ZengWileyCase Reports in Medicine1687-96271687-96352017-01-01201710.1155/2017/57970415797041Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still NeededUroosa Ibrahim0Shiksha Kedia1Gwenalyn Garcia2Jean Paul Atallah3Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USADepartment of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USADepartment of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USADepartment of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USAAntiphospholipid 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-β2-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.http://dx.doi.org/10.1155/2017/5797041 |
| spellingShingle | Uroosa Ibrahim Shiksha Kedia Gwenalyn Garcia Jean Paul Atallah Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed Case Reports in Medicine |
| title | Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed |
| title_full | Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed |
| title_fullStr | Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed |
| title_full_unstemmed | Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed |
| title_short | Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed |
| title_sort | antiphospholipid syndrome multiple manifestations in a single patient a high suspicion is still needed |
| url | http://dx.doi.org/10.1155/2017/5797041 |
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