A Rare Case of Acquired Thrombotic Thrombocytopenic Purpura Triggered by Acute Pancreatitis

Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening hematologic disorder characterized by hemolytic anemia, thrombocytopenia, renal failure, fever, and neurologic dysfunction. While cases often do not present with all five characteristics (<5%), TTP can be hereditary or acquired, often due to a deficiency or dysfunction of the ADAMST13 enzyme. Here, we describe a case of infection-induced acquired TTP in a middle-aged male with urinary tract infection (UTI) and perianal abscess. Suspicion arose from hematologic abnormalities, fever, thrombocytopenia, acute renal failure, and the presence of an underlying infection. A PLASMIC score of 6 (indicating a 72% probability of ADAMTS13 deficiency) prompted ADAMTS13 level testing, revealing levels <5% with the presence of an inhibitor, confirming TTP diagnosis. Treatment with high-dose steroids and daily plasma exchange yielded a swift platelet response, necessitating only two to three days of plasma exchange. In addition, incision and drainage of the perianal abscess were performed. The patient was discharged on daily prednisone and initiated on four doses of weekly Rituximab to mitigate recurrence risk. This case underscores the importance of early suspicion and treatment in infectious triggers such as UTI/perianal abscess, offering crucial diagnostic and prognostic insights.

Section: Discussionmentioning

confidence: 98%

“…It can be inherited or acquired, just as in the case of our patient. An estimated three cases per one million adults per year [2]. It is more common in adults compared to the closely related hematologic disorder, a hemolytic uremic syndrome more common in children [1].…”

Section: Introductionmentioning

confidence: 99%

Acquired Thrombotic Thrombocytopenic Thrombocytopenia Presenting As Urinary Tract Infection and Perianal Abscess: A Case Review

Tolu-Akinnawo,

Oyeleye,

Nnamani

2024

“…TTP can present as a primary disease or can be provoked by a body stressor, including autoimmune disease, infection, malignancy, and pregnancy [ 1 ]. aTTP is classically triggered by sepsis, toxins, and autoimmune disease among others, but not much is known about the association between DKA and aTTP [ 1 , 3 , 5 - 7 ]. DKA can commonly present with electrolyte abnormalities, shock, cerebral edema, and other correctable abnormalities.…”

Section: Discussionmentioning

confidence: 99%

“…Due to the high mortality associated with TTP, it must be suspected in the setting of thrombocytopenia occurring concurrently with hemolytic anemia so that prompt treatment with plasmapheresis can be initiated [ 1 , 5 , 6 ]. TTP is classically triggered by sepsis, toxins, autoimmune disease, radiation, transplant, malignancy, pancreatitis, or cardiovascular surgery, and is reported in a few cases of diabetic ketoacidosis (DKA) [ 1 , 3 , 5 - 7 ]. Here, we report the development of TTP in a man who presented with new-onset diabetes and DKA.…”

Section: Introductionmentioning

confidence: 99%

Diabetic Ketoacidosis: Possible Cause of Thrombotic Thrombocytopenic Purpura

Jackson¹,

Fischer²,

Abdelsayed³

et al. 2021

Acquired thrombotic thrombocytopenic purpura (TTP) is an uncommon microangiopathic disorder that can have variable presentations and can be precipitated by a multitude of stressors to the body, most commonly sepsis. TTP is caused by a deficiency of ADAMTS13 leading to intravascular clotting causing thrombocytopenia and microangiopathic hemolytic anemia. TTP can be associated with various other pathologic conditions. One such rare association has been reported with diabetic ketoacidosis (DKA). Here, we present an even less appreciated presentation in association with DKA. A 62-year-old African American male with previously diagnosed prediabetes presented with DKA and developed hemodynamically significant bleeding. He was confirmed to have TTP that responded to plasmapheresis. TTP is a life-threatening illness if not treated urgently with plasmapheresis with or without rituximab. As acquired TTP most commonly occurs during stress on the body, it is important to treat the underlying stressor. Early identification and initiation of appropriate interventions are crucial to reducing mortality associated with TTP. Furthermore, we need to appreciate less commonly associated conditions such as DKA among patients.

“…TTP is a group of disorders characterized by microvascular thrombosis, hemolytic anemia, and thrombocytopenia. TTP can be caused by a variety of conditions, including infections, medications, malignancies, and autoimmune disorders [ 3 ]. In this case report, we present a rare case of TTP that occurred in a patient with DKA.…”

Section: Introductionmentioning

confidence: 99%

Diabetic Ketoacidosis Complicated by Thrombotic Thrombocytopenic Purpura: A Rare Association

Nwankwo¹,

Samuels²,

Abung³

et al. 2023

Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially life-threatening blood disorder caused by a deficiency or dysfunction of ADAMTS13 and can occur secondary to various conditions, including autoimmune diseases, infections, medications, pregnancy, and malignancies. Diabetic ketoacidosis (DKA) inducing TTP is uncommon and not widely reported in the literature. Herein, we report a case of TTP induced by DKA in an adult patient. His clinical picture, serological, and biochemical results confirmed the diagnosis of TTP induced by DKA, and his clinical course did not improve despite normalization of glucose level, plasmapheresis, and aggressive management. Our case report emphasizes the importance of considering TTP as a potential complication of DKA.