Limits...
An improbable and unusual case of thrombotic thrombocytopenia purpura

View Article: PubMed Central - PubMed

ABSTRACT

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.

No MeSH data available.


ADAMTS13 activity from initial encounter to 45 days after discharge.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC5016747&req=5

Figure 0002: ADAMTS13 activity from initial encounter to 45 days after discharge.

Mentions: 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 150×103/mcL (Fig. 1). 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. Her platelet count at the time of discharge was 338×103/mcL. 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. 2).


An improbable and unusual case of thrombotic thrombocytopenia purpura
ADAMTS13 activity from initial encounter to 45 days after discharge.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5016747&req=5

Figure 0002: ADAMTS13 activity from initial encounter to 45 days after discharge.
Mentions: 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 150×103/mcL (Fig. 1). 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. Her platelet count at the time of discharge was 338×103/mcL. 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. 2).

View Article: PubMed Central - PubMed

ABSTRACT

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.

No MeSH data available.