Altaf Faryal, Shrivastava Shitij, Allena Rachana, Kumar Jai, Khaja Misbahuddin
Internal Medicine, Icahn School of Medicine at Mount Sinai/Bronx Care Health System, New York, USA.
Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.
Cureus. 2024 Apr 10;16(4):e57994. doi: 10.7759/cureus.57994. eCollection 2024 Apr.
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder typically presenting with a classic pentad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal dysfunction, and fever. This report explores an unusual presentation of TTP in a 47-year-old female with a medical history of hypertension, hyperlipidemia, and chronic TTP, who exhibited only petechial rashes, generalized weakness, and headache. Notably, the petechial rash, a less common manifestation of TTP, became a pivotal clue for the diagnosis, underscoring the necessity for vigilance even when classic symptoms are absent. This case reinforces the imperative of a high suspicion index for TTP, especially in patients with thrombocytopenia and hemolytic anemia, irrespective of other traditional signs. Plasmapheresis remains the treatment cornerstone, removing autoantibodies and replenishing ADAMTS13, as evidenced by the patient's initial response. The administration of rituximab, targeting B cells to mitigate autoantibody production against ADAMTS13, featured prominently in her management, aligning with its recognized role in refractory or relapsing TTP cases. Despite an encouraging response to rituximab, a subsequent decline in platelet count indicated the unpredictable nature of TTP and the necessity for multi-pronged therapeutic strategies. The patient's medical background and persistently low ADAMTS13 levels hinted at a chronic relapsing trajectory associated with increased morbidity and mortality. This necessitates ongoing vigilance and treatment flexibility. Highlighting this atypical TTP presentation, the report calls for immediate, robust intervention, serving as a critical reminder of the heterogeneity of TTP manifestations and the complexities in its management, thereby contributing to broader clinical awareness and improved patient prognoses.
血栓性血小板减少性紫癜(TTP)是一种罕见的、危及生命的疾病,通常表现为典型的五联征症状:血小板减少、微血管病性溶血性贫血、神经异常、肾功能不全和发热。本报告探讨了一名47岁女性TTP的不寻常表现,该女性有高血压、高脂血症和慢性TTP病史,仅表现为瘀点皮疹、全身无力和头痛。值得注意的是,瘀点皮疹作为TTP较不常见的表现,成为诊断的关键线索,强调了即使没有典型症状也需保持警惕的必要性。该病例强化了对TTP保持高度怀疑指数的必要性,尤其是在血小板减少和溶血性贫血患者中,无论是否有其他传统体征。血浆置换仍然是治疗的基石,可去除自身抗体并补充ADAMTS13,患者的初始反应证明了这一点。利妥昔单抗的使用,其靶向B细胞以减少针对ADAMTS13的自身抗体产生,在她的治疗中发挥了重要作用,这与其在难治性或复发性TTP病例中的公认作用一致。尽管对利妥昔单抗有令人鼓舞的反应,但随后血小板计数下降表明TTP具有不可预测性,以及采取多管齐下治疗策略的必要性。患者的病史和持续低水平的ADAMTS13提示了与发病率和死亡率增加相关的慢性复发轨迹。这需要持续的警惕和治疗灵活性。该报告强调了这种非典型TTP表现,呼吁立即进行有力干预,这是对TTP表现异质性及其管理复杂性的重要提醒,从而有助于提高更广泛的临床认识和改善患者预后。