Catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia presenting with adrenal insufficiency caused by bilateral hemorrhagic adrenal infarction during sepsis

Abstract Context Coronavirus disease 2019 (COVID-19) is a proinflammatory and prothrombotic condition, but its impact on adrenal function has not been adequately evaluated. Case report A 46-year-old woman presented with abdominal pain, hypotension, skin hyperpigmentation after COVID-19 infection. The patient had hyponatremia, serum cortisol <1.0 ug/dL, ACTH of 807 pg/mL and aldosterone <3 ng/dL. Computed tomography (CT) findings of adrenal enlargement with no parenchymal and minimal peripheral capsular enhancement after contrast were consistent with bilateral adrenal infarction. The patient had autoimmune hepatitis and positive antiphospholipid antibodies, but no previous thrombotic events. The patient was treated with intravenous hydrocortisone, followed by oral hydrocortisone and fludrocortisone. Discussion Among 115 articles, we identified nine articles, including case reports, of new-onset adrenal insufficiency and/or adrenal hemorrhage/infarction on CT in COVID-19. Adrenal insufficiency was hormonally diagnosed in five cases, but ACTH levels were measured in only three cases (high in one case and normal/low in other two cases). Bilateral adrenal non- or hemorrhagic infarction was identified in five reports (two had adrenal insufficiency, two had normal cortisol levels and one case had no data). Interestingly, the only case with well-characterized new-onset acute primary adrenal insufficiency after COVID-19 had a previous diagnosis of antiphospholipid syndrome. In our case, antiphospholipid syndrome diagnosis was established only after the adrenal infarction triggered by COVID-19. Conclusions Our findings support the association between bilateral adrenal infarction and antiphospholipid syndrome triggered by COVID-19. Therefore, patients with positive antiphospholipid antibodies should be closely monitored for symptoms or signs of acute adrenal insufficiency during COVID-19.

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CONCURRENT CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME AND HEPARIN-INDUCED THROMBOCYTOPENIA

CHEST Journal ◽

10.1016/j.chest.2019.08.180 ◽

2019 ◽

Vol 156 (4) ◽

pp. A90-A91 ◽

Cited By ~ 1

Author(s):

Brian Holahan ◽

Kareem Godil ◽

Nathan Brewster ◽

Jonaid Aslam

Keyword(s):

Antiphospholipid Syndrome ◽

Catastrophic Antiphospholipid Syndrome ◽

Heparin Induced Thrombocytopenia

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A “Catastrophic” Heparin-Induced Thrombocytopenia

Case Reports in Medicine ◽

10.1155/2020/6985020 ◽

2020 ◽

Vol 2020 ◽

pp. 1-3 ◽

Cited By ~ 3

Author(s):

D. Barcellona ◽

M. Melis ◽

G. Floris ◽

A. Mameli ◽

A. Muroni ◽

...

Keyword(s):

Antiphospholipid Syndrome ◽

Bleeding Complications ◽

Catastrophic Antiphospholipid Syndrome ◽

Platelet Factor ◽

Cerebral Vessel ◽

Heparin Induced Thrombocytopenia ◽

Unfractioned Heparin ◽

Platelet Protein ◽

Pathological Conditions ◽

Life Threatening

Background. Heparin-induced thrombocytopenia (HIT) is a transient, antibody-mediated thrombocytopenia syndrome that usually follows exposure to unfractioned heparin (UFH) or low-molecular-weight heparin (LMWH). In contrast to other pathological conditions which lead to thrombocytopenia and bleeding complications, HIT results in a paradoxical prothrombotic state. It is caused by antibodies directed to complexes containing UFH or LMWH and a self-platelet protein: the platelet factor 4 (PF4). The heparin-PF4 immune complex leads to activation of platelets, monocytes, and endothelial cells which release procoagulant proteins and tissue factor with subsequent blood coagulation activation. Case Report. We describe the case of a woman undergone to knee replacement and affected by urosepsis who developed a HIT after exposure to enoxaparin. The thrombotic burden was very impressive involving the arterial and venous cerebral vessel and the venous pulmonary, hepatic, and inferior legs vascular beds. The patient was successfully treated with fondaparinux without recurrent thrombosis or bleeding. The clinical scenario could be named “catastrophic HIT” like the catastrophic antiphospholipid syndrome since they have a similar pathogenetic mechanism involving both platelets and monocytes procoagulant activities and a similar clinical manifestation with a life-threatening multiple arterial and/or venous thromboses. Conclusion. Patients presenting with HIT could show a very impressive thrombotic burden resembling to that of the catastrophic antiphospholipid syndrome. A careful differential diagnosis should be made towards other pathological conditions which lead to thrombocytopenia to avoid an unnecessary and potentially harmful platelet transfusion. Although fondaparinux is off-label, its use in patients with HIT is simple and seems to be effective.

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A case-report of successful use of thromboelastography to manage anticoagulation therapy in a patient with concomitant catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia

Blood Coagulation & Fibrinolysis ◽

10.1097/mbc.0000000000000810 ◽

2019 ◽

Vol 30 (4) ◽

pp. 171-175 ◽

Cited By ~ 2

Author(s):

Brian Nguyen ◽

Maria del Mar Morales Hernandez ◽

Cherisse Baldeo ◽

James Cury ◽

Jason A. Ferreira

Keyword(s):

Case Report ◽

Antiphospholipid Syndrome ◽

Anticoagulation Therapy ◽

Catastrophic Antiphospholipid Syndrome ◽

Heparin Induced Thrombocytopenia

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Primary Adrenal Insufficiency Due to Bilateral Adrenal Hemorrhage-Adrenal Infarction in the Antiphospholipid Syndrome: Long-Term Outcome of 16 Patients

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jc.2012-4300 ◽

2013 ◽

Vol 98 (8) ◽

pp. 3179-3189 ◽

Cited By ~ 31

Author(s):

Isolde Ramon ◽

Alexis Mathian ◽

Anne Bachelot ◽

Baptiste Hervier ◽

Julien Haroche ◽

...

Keyword(s):

Adrenal Insufficiency ◽

Acute Phase ◽

Antiphospholipid Syndrome ◽

Adrenal Hemorrhage ◽

Primary Adrenal Insufficiency ◽

Term Outcome ◽

Long Term Outcome ◽

Bilateral Adrenal Hemorrhage ◽

Adrenal Infarction

Context: Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction is a rare and life-threatening manifestation of the antiphospholipid syndrome (APLS). Data on the long-term outcome are scarce. Objective: The aims of the present study were to analyze the long-term outcome related to APLS per se and to characterize the course of adrenal involvement. Design: We conducted a retrospective study of patients with bilateral adrenal hemorrhage-adrenal infarction secondary to APLS seen in the Department of Internal Medicine of Pitié-Salpêtrière Hospital in Paris (France) between January 1990 and July 2010. Results: Three patients died during the acute phase related to APLS manifestations. Sixteen patients (7 males; 9 females) were followed up during a median period of 3.5 years (range 0.3–28.1 years). Three episodes of recurrent thrombosis were noted. One patient died from cerebral hemorrhage 3 months after the onset of adrenal insufficiency. Repeated Synacthen tests showed complete absence of response in 8 of the 10 patients assessed; cortisol and aldosterone increased appropriately in one patient and to some extent in another one. Dehydroepiandrosterone levels and 24-hour urinary epinephrine levels remained abnormally low in all evaluated patients. Adrenal imaging performed more than 1 year after the initial event revealed completely atrophic glands in 9 of 11 patients. Conclusions: This particular subset of APLS patients who survive the acute phase has a rather favorable long-term outcome. Although adrenal dysfunction is generally irreversible, adrenocortical function may, at least partially, recover in rare cases. In this view, measurement of early morning cortisol during follow-up is indicated to detect these patients.

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