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36-year-old female with catastrophic antiphospholipid syndrome treated with eculizumab: a case report and review of literature.

Strakhan M, Hurtado-Sbordoni M, Galeas N, Bakirhan K, Alexis K, Elrafei T - Case Rep Hematol (2014)

Bottom Line: It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation.Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%.The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology and Oncology, Jacobi Medical Center Affiliate of Albert Einstein College of Medicine, 1400 Pelham Parkway, Bronx, NY 10461, USA.

ABSTRACT
Catastrophic antiphospholipid syndrome (CAPS) is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL). CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor.

No MeSH data available.


Related in: MedlinePlus

MRI brain on presentation.
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Related In: Results  -  Collection


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fig1: MRI brain on presentation.

Mentions: Patient was admitted to the medical intensive care unit for close monitoring and blood pressure control. Further workup revealed lactate dehydrogenase levels (LDH) to be significantly elevated at 3,720 U/L and reticulocytosis of 4.2%. Repeat hemoglobin day after admission was noted to be 9.6 g/dL, which was deemed to be secondary to intravenous hydration of a previously dehydrated patient. Peripheral blood smear was significant for 5+ schistocytes per high power field with decreased absolute platelet count and large platelets, strongly suggestive of microangiopathic hemolytic anemia. These findings were concerning for thrombotic thrombocytopenic purpura, prompting initiation of plasmapheresis. After initial lack of response to plasmapheresis with fresh frozen plasma, patient was switched to cryosupernatant and received a total of 15 treatments of pheresis leading to improvement of her platelet count. Further hematologic workup revealed an ADAMTS13 of 58% (reference range: 68%–163%), eliminating thrombotic thrombocytopenic purpura as an etiology, and plasmapheresis was discontinued. Lupus anticoagulant (LA) drawn before initiation of plasmapheresis was reported as positive (18.6 sec, positive test: ≥8 second delta). Patient tested negative for cardiolipin antibody and beta-glycoprotein (<9 SGU). Workup for autoimmune disorder was unrevealing, demonstrating negative comprehensive antinuclear antibody (ANA) panel as well as normal complement levels. Malignant hypertension (HTN) was ruled out with negative metanephrines and renal ultrasound with no evidence of renal artery stenosis. Repeat LA testing was performed and was reported negative (6.6 sec) one month after first positive result. It was believed to have been a false negative result at the time as antiphospholipid antibodies may be removed with plasmapheresis. LA was repeated again at 7 weeks after plasmapheresis, at this point resulting positive (10 seconds), which was confirmed again several days afterwards. Magnetic resonance imaging of the brain (MRI) demonstrated infarction of the right parietooccipital region, left occipital polar region, and the right cerebellar hemisphere consistent with catastrophic antiphospholipid syndrome (Figure 1). Repeat MRI was notable for petechial hemorrhagic conversion of infarct and anticoagulation was deferred at this time as per neurology recommendations. Patient was started on hemodialysis due to her persistent renal failure. Renal biopsy was performed reporting evidence of both acute and chronic thrombotic microangiopathy involving both glomeruli and arteries/arterioles favoring diagnosis of antiphospholipid antibody syndrome (Figure 2). Patient's clinical features were consistent with CAPS (including multiple strokes, non-ST elevation myocardial infarction, end stage renal disease, intraretinal hemorrhage, renal biopsy with multiple arterial thrombi, and positive lupus anticoagulant).


36-year-old female with catastrophic antiphospholipid syndrome treated with eculizumab: a case report and review of literature.

Strakhan M, Hurtado-Sbordoni M, Galeas N, Bakirhan K, Alexis K, Elrafei T - Case Rep Hematol (2014)

MRI brain on presentation.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4214168&req=5

fig1: MRI brain on presentation.
Mentions: Patient was admitted to the medical intensive care unit for close monitoring and blood pressure control. Further workup revealed lactate dehydrogenase levels (LDH) to be significantly elevated at 3,720 U/L and reticulocytosis of 4.2%. Repeat hemoglobin day after admission was noted to be 9.6 g/dL, which was deemed to be secondary to intravenous hydration of a previously dehydrated patient. Peripheral blood smear was significant for 5+ schistocytes per high power field with decreased absolute platelet count and large platelets, strongly suggestive of microangiopathic hemolytic anemia. These findings were concerning for thrombotic thrombocytopenic purpura, prompting initiation of plasmapheresis. After initial lack of response to plasmapheresis with fresh frozen plasma, patient was switched to cryosupernatant and received a total of 15 treatments of pheresis leading to improvement of her platelet count. Further hematologic workup revealed an ADAMTS13 of 58% (reference range: 68%–163%), eliminating thrombotic thrombocytopenic purpura as an etiology, and plasmapheresis was discontinued. Lupus anticoagulant (LA) drawn before initiation of plasmapheresis was reported as positive (18.6 sec, positive test: ≥8 second delta). Patient tested negative for cardiolipin antibody and beta-glycoprotein (<9 SGU). Workup for autoimmune disorder was unrevealing, demonstrating negative comprehensive antinuclear antibody (ANA) panel as well as normal complement levels. Malignant hypertension (HTN) was ruled out with negative metanephrines and renal ultrasound with no evidence of renal artery stenosis. Repeat LA testing was performed and was reported negative (6.6 sec) one month after first positive result. It was believed to have been a false negative result at the time as antiphospholipid antibodies may be removed with plasmapheresis. LA was repeated again at 7 weeks after plasmapheresis, at this point resulting positive (10 seconds), which was confirmed again several days afterwards. Magnetic resonance imaging of the brain (MRI) demonstrated infarction of the right parietooccipital region, left occipital polar region, and the right cerebellar hemisphere consistent with catastrophic antiphospholipid syndrome (Figure 1). Repeat MRI was notable for petechial hemorrhagic conversion of infarct and anticoagulation was deferred at this time as per neurology recommendations. Patient was started on hemodialysis due to her persistent renal failure. Renal biopsy was performed reporting evidence of both acute and chronic thrombotic microangiopathy involving both glomeruli and arteries/arterioles favoring diagnosis of antiphospholipid antibody syndrome (Figure 2). Patient's clinical features were consistent with CAPS (including multiple strokes, non-ST elevation myocardial infarction, end stage renal disease, intraretinal hemorrhage, renal biopsy with multiple arterial thrombi, and positive lupus anticoagulant).

Bottom Line: It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation.Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%.The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology and Oncology, Jacobi Medical Center Affiliate of Albert Einstein College of Medicine, 1400 Pelham Parkway, Bronx, NY 10461, USA.

ABSTRACT
Catastrophic antiphospholipid syndrome (CAPS) is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL). CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor.

No MeSH data available.


Related in: MedlinePlus