a 54-year - old female presented to the emergency department with complaints of gingival bleeding , hematuria , and bilateral lower extremity petechial rash that began 2 days prior to admission . her symptoms started with gross hematuria for which she was evaluated at a prompt care and started on ciprofloxacin for a presumed urinary tract infection . the following day , she experienced gingival bleeding while brushing her teeth and a petechial rash on her lower extremities , prompting her to undergo further evaluation at the emergency department . her past medical history was significant for idiopathic thrombocytopenic purpura diagnosed in 1995 , ttp and acute cerebrovascular attack diagnosed in 1997 , a relapse of ttp which resulted in a splenectomy in 1999 , hyperthyroidism secondary to grave 's disease , and hyperlipidemia . her social history was negative for any alcohol use and she formerly smoked cigarettes . on physical exam , she was afebrile and had normal vital signs with the exception of tachycardia with a heart rate of 103 . initial laboratory studies were significant for mild anemia and thrombocytopenia with a hemoglobin 11.5 g / dl , hematocrit 34.4% , mcv 84.5 fl , and platelet count 1710/mcl . her ldh was 1236 u / l , her haptoglobin was < 8 mg / dl , and her reticulocyte count was 2.3% . her chemistry panel and liver enzymes were unremarkable with the exception of a total bilirubin of 2.4 mg / dl and a direct bilirubin of 0.7 mg / dl . corticosteroid therapy was promptly initiated , and hematology and nephrology were consulted for presumed ttp . an adamts13 activity level was drawn and sent for evaluation . due to her grave 's disease , thyroid peroxidase antibody and tsh were evaluated and found to be 180 iu / ml and < 0.010 miu / l , respectively . she was asymptomatic and requested outpatient follow - up with her endocrinologist for the initiation of hyperthyroidism treatment . without delay , she underwent temporary dialysis catheter placement and began therapeutic plasma exchange ( tpe ) . her platelet counts immediately improved following her first session of tpe , and she continued daily tpe with corticosteroid therapy until her platelet count was maintained greater than 15010/mcl ( fig . her adamts13 activity level resulted on day 4 after the admission and was decreased at 13% . her adamts13 inhibitor was found to be positive . in total , she received 5 days of tpe and corticosteroid therapy before ceasing tpe and initiating a 6-week oral steroid taper . an adamts13 activity was repeated 1 week after her initial draw , which resulted as 43% activity , and again 1 month later , with a value of 56% activity ( fig . ttp is presumptively diagnosed by clinical and diagnostic evaluations with the presence of microangiopathic hemolytic anemia , thrombocytopenia , elevated ldh , and bilirubin with negative coombs testing . the diagnosis is definitive with the presence of decreased adamts13 activity , with levels less than 10% . in the cases reviewed by griffin et al . , the differential diagnosis considered in patients with ttp prior to the diagnosis of ttp included meningitis , urosepsis , immune thrombocytopenia , stroke , systemic lupus erythematosus , gastroenteritis , arthritis , and stroke to name a few ( 6 ) . owing to its high fatality rate , treatment is initiated with a presumptive diagnosis of ttp , prior to obtaining the results from more confirmative testing such as the adamts13 levels . prior to the 1980s , before the era of therapeutic plasma exchange for treatment of ttp , fatality rates were greater than 90% ( 7 , 8) . treatment consists of prompt and daily therapeutic plasma exchange and glucocorticoid administration , which may be oral or intravenous depending on the patient 's neurological status . once the platelet count is maintained above 150,000/l for 2 days , therapeutic plasma exchange can be discontinued while continuing steroid therapy . as the platelet count plateaus if patients have a recurrence of ttp within 1 month , they have refractory disease and will need to undergo retreatment with plasma exchange , glucocorticoids , and may additionally be treated with rituximab . if the patient remains recurrence free for 1 month , they are in remission . if the recurrence occurs after 1 month , they have relapsing ttp and must also repeat therapy as mentioned for refractory disease and may also require the use of rituximab or splenectomy . rituximab , a monoclonal antibody against cd20 , has been shown to be effective in treating refractory disease in acquired tpp 911 . one study found that the relapse rate was approximately 36% within a 30-month follow - up period and approximately 76% of those occurred within the first 24 months ( 13 ) . in patients with relapsing or refractory disease , splenectomy remains a viable , but not curative , treatment option . in a case series by dubois and gray , patients who underwent splenectomy for relapsing ttp fared better than those who underwent splenectomy for refractory disease . they noted the aggregate complication rate to be 6% versus 10% , and mortality rate to be 1.2% versus 5% in the relapsing ttp group that underwent splenectomy versus the refractory group . furthermore , they found the post - splenectomy relapse rate for ttp was approximately 17% ( 14 ) . due to the risk of significant morbidity and mortality that may result from delayed treatment of ttp as well as the significant variations of presentation , ttp requires a consistently high index of suspicion . additionally , once a patient has been diagnosed with ttp , the rate of relapse is 40% , which may be decreased to approximately 17% after splenectomy . our patient suffered multiple relapses within a 30-year span , underwent splenectomy in early adulthood , and presented with atypical symptoms during her most recent relapse illustrating how persistent ttp can be as well as how unusually it may present . ttp should be considered as part of the differential for any patient presenting with anemia and thrombocytopenia , regardless of lack of other symptoms as they may often be absent . additionally , we recommend that patients who have experienced ttp receive education on relapsing and refractory disease , as well as typical and atypical symptoms that may indicate a recurrence . lastly , we recommend educating patients to inform their provider of their history of ttp early in their diagnostic process should there be concern for a recurrence . jp and pp reviewed the literature , drafted the manuscript , and approved the final version of the article to be published . za reviewed the literature , made critical revisions related to the content of the article , and approved the final version of the article to be published . the study was supported by the university of illinois peoria campus , osf saint francis medical center . the views expressed in the submitted article are our own and not an official position of the institution .
thrombotic thrombocytopenic purpura ( ttp ) is a life - threatening medical emergency which may be difficult to recognize given the wide spectrum in which it presents . 
 a delay in treatment may be catastrophic as untreated cases of ttp have a mortality rate exceeding 90% . 
 given the high fatality rate of untreated ttp and its range of presenting symptoms , we present our unusual case of ttp in a post - splenectomy patient with early treatment and positive outcome . 
 this case describes a 54-year - old female who presented with hematuria and gingival bleeding , followed by the development of a bilateral lower extremity petechial rash . 
 her past medical history was significant for multiple episodes of ttp , the last of which resulted in a splenectomy and a 20-year history of remission thereafter . on exam , she was alert , well appearing , and neurologically intact . 
 her only significant finding was a bilateral lower extremity petechial rash . 
 laboratory studies revealed mild anemia and thrombocytopenia , an elevated lactate dehydrogenase , and a decreased haptoglobin . 
 peripheral smear showed poikilocytosis , helmet cells , and schistocytes . 
 corticosteroid therapy was promptly initiated , her platelets were monitored closely , and she underwent urgent therapeutic plasma exchange . due to the risk of significant morbidity and mortality that may result from delayed treatment of ttp as well as the significant variations of presentation , ttp requires a consistently high index of suspicion . 
 our patient suffered multiple relapses of ttp within a 30-year span , underwent splenectomy in early adulthood , and presented with atypical symptoms during her most recent relapse illustrating how persistent ttp can be as well as how unusually it may present . 
 providers should be aware of the vast spectrum of presentation and remember that ttp may recur following splenectomy despite prolonged remission .