Spontaneous HIT syndrome clinically/serologically resembles HIT but without proximate heparin.
Rarely, spontaneous HIT syndrome complicates total knee arthroplasty surgery.
Mesenteric vein thrombosis is a rare presentation of spontaneous HIT syndrome.
IVIg rapidly corrects thrombocytopenia by inhibiting heparin‐independent platelet activation.
Summary
Spontaneous heparin‐induced thrombocytopenia (HIT) syndrome is an autoimmune HIT (aHIT) disorder characterized by thrombocytopenia, thrombosis, and HIT antibodies despite no proximate heparin exposure. For unknown reasons, many cases occur after total knee arthroplasty. A 52‐year‐old woman presented 12 days posttotal knee replacement (aspirin thromboprophylaxis) with gastrointestinal bleeding (superior mesenteric vein thrombosis); the platelet count was 63 × 109 L−1. After bowel resection and a brief course of heparin, treatment was changed to argatroban followed by fondaparinux. In addition, high‐dose intravenous immunoglobulin (IVIg), 1 g kg−1 on 2 consecutive days, resulted in abrupt platelet count rise from 21 (nadir) pre‐IVIg to 137 (post‐IVIg), and 2 days later to 200 × 109 L−1. Heparin‐independent serum‐induced serotonin‐release abruptly decreased from 91% (pre‐IVIg) to 14% (post‐IVIg); although serotonin‐release later rebounded to 49%, the patient's platelet counts remained normal. Our observations support the emerging concept that high‐dose IVIg is effective for treating aHIT disorders, including spontaneous HIT syndrome.
Acute Generalized Exanthematous Pustulosis (AGEP) is a rare drug reaction manifesting as pustular lesions with surrounding erythema following exposure. The disease is often self-limited and treatment is supportive. It may present in an atypical variant with vesicles that desquamate into erosions, which classifies the disease as an AGEP/SJS Overlap. This overlap syndrome can carry a substantial mortality rate and necessitate elevation in the level of care. Hydroxychloroquine has been implicated in cases of AGEP, and we present a case of AGEP/SJS overlap attributed to this common medication. Given the prevalence of drug eruptions, it is critical for the physicians to recognize and not overlook this rare and potentially fatal dermatological emergency.
Apixaban is a direct oral anticoagulant that works by inhibiting factor Xa. It has been associated with adverse bleeding outcomes including atraumatic splenic rupture. We present the case of an 86-year-old man who presented with features of left upper abdominal pain and hemorrhagic shock found to have atraumatic splenic rupture and hemoperitoneum on imaging.
We present a case of a 79-year-old male who presented with retroperitoneal hematoma a week after motor vehicle accident. Prior history and family history of bleeding were nonsignificant. His activated partial thromboplastin time was found to be prolonged in the emergency department. Further workup with coagulation studies showed decreased factor VIII, vWF antigen, and vWF:ristocetin cofactor assay, and negative Bethesda assay, indicating acquired von Willebrand disease. Immunofluorescence to find an underlying etiology was suggestive of MGUS. Management of AvWD depends on controlling active bleeding and treating the underlying cause. He was treated with factor VIII, haemate-p, rituximab, two cycles of IVIg, and three weeks of oral steroids.
Pulmonary mucormycosis is a rare life-threatening fungal infection associated with high mortality. We present the case of a 61-year-old man with history of chronic lymphocytic leukemia who presented with fever and cough, eventually diagnosed with pulmonary mucormycosis after right lung video-assisted thoracoscopic surgery. The patient was successfully treated with amphotericin B and right lung pneumonectomy; however, he later died from left lung pneumonia.
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