Adolescent ; Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Male ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; etiology ; therapy

We report on a patient with systemic lupus erythematosus, who, during the course of the illness, developed thrombotic thrombocytopenic purpura. In this case, the coexistence of these two conditions was confirmed by laboratory and pathologic findings. The infusion of fresh frozen plasma with plasmapheresis reversed the course of thrombotic thrombocytopenic purpura.
Adult ; *Blood Transfusion ; Combined Modality Therapy ; Humans ; Lupus Erythematosus, Systemic/*complications ; Male ; *Plasma ; *Plasmapheresis ; Purpura, Thrombotic Thrombocytopenic/etiology/*therapy ; Syndrome

Although thrombotic thrombocytopenic purpura (TTP) is rarely seen in pediatric patients, failure to recognize this condition often leads to severe consequences and poor outcomes. Classic features of TTP include thrombocytopenia, microangiopathic hemolytic anemia, acute kidney injury, fever, and central nervous system involvement. However, patients suffering from this condition may not present with all of the symptoms simultaneously. Therefore, it is of utmost importance for healthcare providers to have a high index of suspicion. Laboratory investigations may reveal the presence of schistocytes on peripheral blood smear, negative Coombs test, high lactate dehydrogenase levels and severely low platelet counts. The etiology of TTP is mainly due to insufficient cleavage of the large multimers of von Willebrand factor (vWF) secondary to decreased activity of ADAMTS13 (a disintegrin and metalloprotease with Thrombospondin type 1 repeats, member 13). TTP can be broadly classified into familial TTP (Upshaw Schulman syndrome) and non-familial TTP. Familial TTP is due to a congenital deficiency of ADAMTS13. Its mainstay of therapy is initiation of plasmapheresis during the acute phase, followed by regular fresh frozen plasma (FFP) infusions. Alternatively, non-familial TTP is due to a decrease in ADAMTS13 activity secondary to the presence of anti-ADAMTS13 antibodies. Once again, the primary treatment is plasmapheresis; however, recent anecdotal data also supports the use of rituximab in select cases.
ADAM Proteins ; genetics ; ADAMTS13 Protein ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Child ; Humans ; Plasmapheresis ; Purpura, Thrombotic Thrombocytopenic ; etiology ; therapy ; Rituximab

Acute Kidney Injury ; etiology ; Adult ; Diagnosis, Differential ; Female ; HELLP Syndrome ; pathology ; Hemolytic-Uremic Syndrome ; complications ; pathology ; therapy ; Humans ; Plasma Exchange ; Postpartum Period ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic ; pathology ; Young Adult

Androgen Antagonists ; therapeutic use ; Anilides ; therapeutic use ; Antineoplastic Agents, Hormonal ; therapeutic use ; Bone Neoplasms ; complications ; secondary ; Goserelin ; therapeutic use ; Humans ; Male ; Middle Aged ; Nitriles ; therapeutic use ; Prostatic Neoplasms ; complications ; drug therapy ; Purpura, Thrombotic Thrombocytopenic ; drug therapy ; etiology ; Tosyl Compounds ; therapeutic use