TY - JOUR
T1 - Differentiating malignant hypertension-induced thrombotic microangiopathy from thrombotic thrombocytopenic purpura
AU - Khanal, Nabin
AU - Upadhyay, Smrity
AU - Dahal, Sumit
AU - Bhatt, Vijaya Raj
AU - Bierman, Philip J.
PY - 2015/6
Y1 - 2015/6
N2 - Malignant hypertension can cause thrombotic microangiopathy (TMA) and the overall presentation may mimic thrombotic thrombocytopenic purpura (TTP). This presents a dilemma of whether or not to initiate plasma exchange. The objective of the study was to determine the clinical and laboratory manifestations of malignant hypertension-induced TMA, and its outcomes. Methods: Using several search terms, we reviewed English language articles on malignant hypertension-induced TMA, indexed in MEDLINE by 31 December 2013. We also report a new case. All these cases were analyzed using descriptive statistics. Results: A total of 19 patients, with 10 males, had a median age of 38 years at diagnosis; 58% had a history of hypertension. Mean arterial pressure at presentation was 159mmHg (range 123-190mmHg). All had prominent renal dysfunction (mean creatinine of 5.2mg/dl, range 1.7-13mg/dl) but relatively modest thrombocytopenia (mean platelet count of 60-103/Al, range 12-131-103/Al). Reported cases (n=9) mostly had preserved ADAMTS-13 activity (mean 64%, range 18-96%). Following blood pressure control, the majority had improvement in presenting symptoms (100%) and platelet counts (84%); however, only 58% had significant improvement in creatinine. More than half (53%) needed hemodialysis. One patient died of cardiac arrest during pacemaker insertion. Prior history of hypertension, high mean arterial pressure, significant renal impairment but relatively modest thrombocytopenia and lack of severe ADAMTS-13 deficiency (activity <10%) at diagnosis are clues to diagnose malignant hypertension-induced TMA. Patients with malignant hypertension respond well to antihypertensive agents and have favorable nonrenal outcomes.
AB - Malignant hypertension can cause thrombotic microangiopathy (TMA) and the overall presentation may mimic thrombotic thrombocytopenic purpura (TTP). This presents a dilemma of whether or not to initiate plasma exchange. The objective of the study was to determine the clinical and laboratory manifestations of malignant hypertension-induced TMA, and its outcomes. Methods: Using several search terms, we reviewed English language articles on malignant hypertension-induced TMA, indexed in MEDLINE by 31 December 2013. We also report a new case. All these cases were analyzed using descriptive statistics. Results: A total of 19 patients, with 10 males, had a median age of 38 years at diagnosis; 58% had a history of hypertension. Mean arterial pressure at presentation was 159mmHg (range 123-190mmHg). All had prominent renal dysfunction (mean creatinine of 5.2mg/dl, range 1.7-13mg/dl) but relatively modest thrombocytopenia (mean platelet count of 60-103/Al, range 12-131-103/Al). Reported cases (n=9) mostly had preserved ADAMTS-13 activity (mean 64%, range 18-96%). Following blood pressure control, the majority had improvement in presenting symptoms (100%) and platelet counts (84%); however, only 58% had significant improvement in creatinine. More than half (53%) needed hemodialysis. One patient died of cardiac arrest during pacemaker insertion. Prior history of hypertension, high mean arterial pressure, significant renal impairment but relatively modest thrombocytopenia and lack of severe ADAMTS-13 deficiency (activity <10%) at diagnosis are clues to diagnose malignant hypertension-induced TMA. Patients with malignant hypertension respond well to antihypertensive agents and have favorable nonrenal outcomes.
KW - ADAMTS-13 deficiency
KW - malignant hypertension
KW - plasma exchange
KW - renal failure
KW - thrombocytopenia
KW - thrombotic microangiopathy
KW - thrombotic thrombocytopenic purpura
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U2 - 10.1177/2040620715571076
DO - 10.1177/2040620715571076
M3 - Article
C2 - 26137201
AN - SCOPUS:84993736115
VL - 6
SP - 97
EP - 102
JO - Therapeutic Advances in Hematology
JF - Therapeutic Advances in Hematology
SN - 2040-6207
IS - 3
ER -