Pasli Melisa, Lovell Katie K, Vulasala Sai Swarupa R, Hairr Marsha L, Bandaru Revanth Reddy, Khalilullah Mohammad Z, Johnson Leonard
Medical School, Brody School of Medicine at East Carolina University, Greenville, USA.
Internal Medicine, East Carolina University Health, Greenville, USA.
Cureus. 2023 Jan 24;15(1):e34135. doi: 10.7759/cureus.34135. eCollection 2023 Jan.
Sarcoidosis is a multisystem inflammatory disorder characterized by the formation of non-caseating granulomas. Hematological manifestations such as thrombocytopenia are unusual presentations of the disease. Various theories have been proposed for the development of thrombocytopenia in patients with sarcoidosis such as decreased production in bone marrow caused by granuloma formation, hypersplenism, and immune thrombocytopenia (ITP). We present a case of a 30-year-old African American male with ITP secondary to sarcoidosis who presented with a sudden onset of buccal mucosa and mucocutaneous bleeding and was found to have severe thrombocytopenia with values reaching as low as 1000/uL without prior history of easy bruising or bleeding. Overall, our patient had dyspnea, mucocutaneous bleeding, and was found to have mediastinal and hilar adenopathy, isolated thrombocytopenia, no splenomegaly, and non-necrotizing granulomas in the lymph nodes. The patient received platelet transfusions without initial response and received intravenous immunoglobulin (IVIG), romiplostim, and steroids with subsequent improvement in the platelet count after sufficient administration of a treatment regimen of approximately one week. Confounding factors that resulted in diagnostic uncertainty of our patient presentation included travel history with prophylactic antimalarial medications, doxycycline usage, only slightly elevated Angiotensin-Converting Enzyme (ACE) levels, and imaging features concerning metastatic disease vs. lymphoma. The clinical diversity of sarcoidosis often leads to diagnostic uncertainty and treatment delays due to its resemblance to other more common disorders. This is a novel case report of the earliest temporal presentation of severe thrombocytopenia and sarcoidosis in an African American male reported in the literature.
结节病是一种多系统炎症性疾病,其特征是形成非干酪样肉芽肿。血小板减少等血液学表现是该疾病不常见的表现形式。关于结节病患者血小板减少的发生提出了各种理论,例如肉芽肿形成导致骨髓生成减少、脾功能亢进和免疫性血小板减少症(ITP)。我们报告一例30岁非裔美国男性病例,该患者因结节病继发ITP,表现为颊黏膜和皮肤黏膜突然出血,发现有严重血小板减少,数值低至1000/μL,既往无易瘀伤或出血史。总体而言,我们的患者有呼吸困难、皮肤黏膜出血,发现有纵隔和肺门淋巴结肿大、孤立性血小板减少、无脾肿大以及淋巴结中有非坏死性肉芽肿。患者接受血小板输注后最初无反应,随后接受静脉注射免疫球蛋白(IVIG)、罗米司亭和类固醇治疗,在给予约一周的治疗方案后血小板计数随后有所改善。导致我们患者临床表现诊断不确定性的混杂因素包括有预防性抗疟药物的旅行史、使用强力霉素、血管紧张素转换酶(ACE)水平仅略有升高以及关于转移性疾病与淋巴瘤的影像学特征。结节病的临床多样性常常因其与其他更常见疾病相似而导致诊断不确定性和治疗延迟。这是文献中报道的首例非裔美国男性最早出现严重血小板减少和结节病的病例报告。