Epidermolysis Bullosa Acquisita in a patient with End Stage Renal Disease: Case study.

The clinical presentation of COVID-19 varies from being asymptomatic to developing acute respiratory distress syndrome and multi-organ dysfunction. The diffuse microvascular thrombi in multiple organs seen in the autopsy of COVID-19 patients are similar to that of thrombotic microangiopathy (TMA). TMA is characterised by thrombus formation in the microvasculature with laboratory findings of microangiopathic haemolytic anaemia (MAHA) and thrombocytopenia. A 49-year-old male presented to the Aga Khan University Hospital, Karachi. with fever, diarrhoea, altered level of consciousness, and a positive nasopharyngeal swab for SARS-CoV-2. He developed severe thrombocytopenia, MAHA with 5.8% schistocytes, and worsening renal function on the sixth day of admission. Diagnosis of thrombotic thrombocytopenic purpura (TTP) was established based on PLASMIC score, and he was successfully treated with intravenous (IV) Methylprednisolone, therapeutic plasma exchange and IV Rituximab. The case emphasises the need to keep TTP in the differential diagnosis when a patient with COVID-19 develops severe thrombocytopenia, acute renal failure, or impaired level of consciousness, since prompt diagnosis and treatment is necessary to gain favourable outcome.

Thrombotic thrombocytopenic purpura as an acute complication of COVID-19.

Introduction: While disseminated intravascular coagulation (DIC) is a serious complication of COVID-19, a close differential in critically ill patients with thrombocytopenia is Thrombotic thrombocytopenic purpura (TTP). Case Report: We describe the case of a middle-aged lady admitted with COVID-19 pneumonia who developed progressive thrombocytopenia, altered sensorium and renal failure. The absence of coagulation abnormalities alerted to the possibility of TTP, strengthened by presence of schistocytes in peripheral smear. Conclusions: This case highlights the need for high index of suspicion and to pay attention to normal tests as well that might give clues to the diagnosis. New onset thrombocytopenia in COVID-19 need not always indicate DIC. A careful examination of peripheral smear may help diagnosing TTP especially if coagulation profile is normal.

Thrombocytopenia: the good, the bad and the ugly.

New thrombocytopenia may be associated with a variety of conditions and diagnosis can be challenging. Presentation can vary from life-threatening bleeding or thrombosis to an incidental finding in an asymptomatic patient. New thrombocytopenia requires urgent investigation. Investigations are mainly guided by findings from the clinical history, physical examination, full blood count and blood film analysis. Aside from the actively bleeding patient, rare but life-threatening causes of thrombocytopenia must be identified early as they require urgent treatment. These include thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, suspicion of new acute promyelocytic leukaemia, and vaccine-induced prothrombotic immune thrombocytopenia. Here, we discuss how to approach a patient with new thrombocytopenia, along with key differentials not to be missed.

Thrombotic thrombocytopenic purpura following administration of the Moderna booster vaccine.

Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy that is characterized by microangiopathic haemolytic anaemia, consumption thrombocytopenia and organ injury. It is caused by a severe deficiency of ADAMTS13, which can be either congenital or acquired. There is a plethora of things that can cause the acquired form, including medications and infections. Vaccines have also been shown to cause TTP. In the midst of the COVID-19 pandemic, with multiple new vaccines being developed and distributed to the masses, the medical community needs to be aware of adverse events associated with these new vaccines. We present a case of TTP following administration of the Moderna booster vaccine.

Unusual Presentation of Thrombotic Thrombocytopenic Purpura in a Newly Diagnosed Pediatric Patient With Systemic Lupus Erythematosus in the Setting of MIS-C.

The understanding of coronavirus disease 2019 (COVID-19) immune dysregulation is evolving. Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with alternations in both innate and adaptive immunity, probably caused by a complex interplay of genetics and environmental exposure with various triggers. A rare hematological complication of SLE as well as recently reported in an adult with COVID-19 is thrombotic thrombocytopenic purpura. We report a pediatric case with features suggestive of the multisystem inflammatory syndrome in children with coronary artery ectasia, thrombotic thrombocytopenic purpura, and new-onset SLE.

Thrombotic thrombocytopenic purpura associated with COVID-19 in a critically ill child: a Peruvian case report.

BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease. In middle and low-income countries, specific resources are required for its diagnosis due to the lack of diagnostic tests and the variable response to plasma exchange, especially in the context of the new SARS-CoV-2 pandemic. CASE REPORT: We report the case of a 9-year-old male Hispanic patient with SARS-CoV-2 infection, atypical presentation, and multisystem involvement, thrombotic microangiopathy with dermal manifestations, hematologic, renal, and neurologic involvement. The patient was followed up after SARS-CoV-2 infection, the PLASMIC score was applied, and a genetic study was performed. Ventilation and hemodynamic support, corticotherapy, immunoglobulins, plasma exchange, renal replacement therapy, and monoclonal antibodies were given without favorable response. CONCLUSIONS: TTP associated with SARS-CoV-2 in the pediatric population is rare. However, resources for the diagnosis, support, and management of patients with TTP are required to avoid fatal outcomes.

INTRODUCCIÓN: La púrpura trombocitopénica trombótica (PTT) adquirida es una enfermedad poco frecuente. En los países de mediano y bajo estatus económico se requieren recursos para el diagnóstico de la PTT, debido a la falta de pruebas diagnósticas y a la respuesta variable al recambio plasmático, especialmente en el contexto de la pandemia por el nuevo SARS-CoV-2. CASO CLÍNICO: Paciente de sexo masculino, de 9 años, hispano, con infección por SARS-CoV-2, presentación atípica y afectación multisistémica, microangiopatía trombótica con manifestaciones dérmicas, y compromiso hematológico, renal y neurológico. Se dio seguimiento posinfección por SARS-CoV-2, se aplicó la escala PLASMIC y se realizó un estudio genético. Se aplicaron soporte ventilatorio y hemodinámico, corticoterapia, inmunoglobulinas, recambio plasmático, terapia de reemplazo renal y anticuerpos monoclonales, sin respuesta favorable. CONCLUSIONES: La PTT asociada al SARS-CoV-2 en la población pediátrica es poco frecuente. Aun así, se requieren recursos para el diagnóstico, el soporte y el manejo de los pacientes con PTT para evitar desenlaces fatales.

Complicated case of COVID-19 disease with overlapping features of thrombotic thrombocytopenic purpura and haemophagocytic lymphohistiocytosis.

Haemophagocytic lymphohistiocytosis has been reported as an uncommon complication of severe COVID-19 disease while thrombotic thrombocytopenic purpura has been rarely reported. Here, we are reporting a 21-year-old man who developed a combination of these complications during the hospital stay in the post-COVID-19 recovery period. He presented with fever and bilateral COVID-19-related pneumonia requiring invasive ventilation. His hospital course was complicated by the development of pneumothorax, ventilator-associated pneumonia, thrombotic thrombocytopenic purpura and haemophagocytic lymphohistiocytosis. He received remdesivir, IVIG, steroid, fresh frozen plasma and supportive care but had a fatal outcome.