Abstract Number: PB1834
Meeting: ISTH 2020 Congress
Background: Thrombocytopenia in sarcoidosis is a rare manifestation essentially secondary to hypersplenism or bone marrow involvement.
Aims: Autoimmune thrombocytopenic purpura (ATP) has been rarely described associated with sarcoidosis.
Methods: we present a new case report.
Results: Mrs. FR, aged 50, with a history of pulmonary tuberculosis 8 years ago, has been followed up for mediastino-pulmonary sarcoidosis with sinusal involvement for 3 years.
The diagnosis was based on cutaneous lesions such as lupus pernio and erythema nodosum of lower extremities, nasal obstruction, mediastinal and intraperitoneal lymphadenopathy and bilateral pulmonary infiltrates, increased angiotensin-converting enzyme (ACE) level and the presence of an epithelioid granuloma at salivary gland biopsy and nasal mucosa. She was treated with oral corticosteroid and the evolution was, initially, favorable.
While the patient was taking 10 mg of Prednisone per day, she presented a great abundance epistaxis resisting to local treatment. Laboratory testing revealed thrombocytopenia at 3000el/mm3 and anemia at 5g / dl of hemoglobin. Myelogram, liver function test, LDH and complement were normal. Anti-nuclear antibodies, anticardiolipin and anti-β2 GP1 were negative. There were no signs of infection. Abdominal ultrasound was normal. The diagnosis of ATP associated with a Sarcoidosis was set.
The patient was treated with tranexamic acid and red blood cells transfusions. Prednisone was increased to 1mg / kg / day. The evolution was marked by a rise in platelet count within 72 hours and nasal bleeding stopped after 24 hours.
Conclusions: Thrombocytopenia in sarcoidosis is explained by three mechanisms: hypersplenism, medullary invasion by granulomas or more rarely an immunological mechanism. ATP occurs during sarcoidosis in less than 2% of cases and is particularly severe, often complicated by hemorrhage and associated with a high mortality rate of up to 15% of patients. Sarcoidosis associated with ATP is characterized by severe systemic involvement and frequent relapses needing prolonged systemic treatment.
To cite this abstract in AMA style:Cherif Y, Mrouki M, Derbel S, Ben Dahmen F, Abdallah M. Sarcoidosis and Autoimmune Thrombocytopenic Purpura: Random Association or Hematologic Manifestation of Sarcoidosis? [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/sarcoidosis-and-autoimmune-thrombocytopenic-purpura-random-association-or-hematologic-manifestation-of-sarcoidosis/. Accessed September 22, 2023.
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