thrombotic thrombocytopenic purpura ( ttp ) is a life - threatening disorder classically presenting with fever , thrombocytopenia , microangiopathic hemolytic anemia , renal dysfunction , and neurological impairment as well as various multiorgan complications and a mortality rate reaching 95% for untreated cases.1 the survival rate is 80%90% with early diagnosis and treatment with plasma infusion and plasma exchange .
a 39-year - old sudanese male presented to the emergency room with fever , jaundice , and decreasing level of consciousness ( inability to recognize relatives ) for 7 days .
the following laboratory values were obtained : white blood cell ( wbc ) count , 8.6  10/l ; hemoglobin ( hb ) , 67 g / l ; platelet ( plt ) count , 8  10/l ; creatinine , 121 mol / l ; prothrombin time and partial thromboplastin time , normal ; lactate dehydrogenase level ( ldh ) , 1947 u / l ; increased reticulocyte count ; and blood smear showing schistocytes .
a urine analysis showed the following : blood , + 4 ; ph 5.5 ; protein , + 2 ; red blood cell count , 1520 ; wbc count , 1 ; and specific gravity , 1.0170 .
the presence of microangiopathic hemolytic anemia , thrombocytopenic purpura , neurologic abnormalities , fever , and renal disease confirmed the patient s diagnosis .
ten days later , his ldh rose to 1270 u / l , with hb of 105 g / l and plt count of 14  10/l .
the patient was also started on cryoprecipitate supernatant because of the suboptimal response to plasmapheresis with fresh frozen plasma .
this was thought to be a relapse of ttp ; the condition was considered and managed as resistant ttp .
two days later , intravenous immunoglobulin ( full dose ) was started . despite the treatment for resistant ttp
, the patient worsened , requiring icu ( intensive care unit ) admission because of the development of uncontrolled seizures , which required intubation and ventilation . the patient developed left - sided weakness and hypotension .
treatment with antibiotics and then antifungal therapy commenced ; the case was labeled as refractory ttp .
repeated brain computed tomography scans were normal . during this process , the creatinine kinase ( ck )
levels increased to > 50,000 u / l , with worsening kidney function and decreased urine output .
acute renal failure in this patient was diagnosed according to the rifle ( risk , injury , failure , loss , end stage ) criteria : a rise in creatinine of more than three - fold , and urine output < 0.3 ml / kg / h over 24 hours .
urine alkalanization was started when creatine phosphokinase ( cpk ) started rising to a target urine ph of 8 , with several fluid boluses targeting a urine output of > 200 ml / h .
the serum calcium concentration was monitored on a daily basis throughout the patient s stay in the icu and after his discharge to a step - down unit .
continuous venovenous hemodialysis started , although the cpk levels did not decrease for 5 days .
after maintaining the patient on dialysis for 2 weeks , the patient started to improve clinically and was more alert , with a ck level of 5360 u / l and plt count of 95  109/l ( see figure 1 ) .
all possible differential diagnoses for rhabdomyolysis were investigated , including metabolism , viral serology , bacterial levels , toxins , and drugs .
the difficulty in weaning the patient from the ventilator stemmed from critical illness polyneuropathy and the consequences of the tracheostomy .
he was conscious and alert , with residual weakness / paresis of the lower limbs , which was managed with physiotherapy .
potential causes include malignant hyperthermia , neuroleptic malignant syndrome , near drowning / hypothermia , hiv ( human immunodeficiency virus ) infection , snake bites , heritable muscle enzyme deficiencies , metabolic and inflammatory myopathies , status epilepticus , status asthmaticus , eclampsia , prolonged labor , alcohol and cocaine abuse , copper sulfate , zinc phosphide , and other drugs .
traumatic and nontraumatic causes of rhabdomyolysis have been described in other reviews.2,3 acute renal failure in rhabdomyolysis can be attributed to many mechanisms , such as precipitated myoglobin that blocks the renal tubules , oxygen free - radical injury , and renal arteriolar vasoconstriction induced by myoglobin .
acidic urine precipitates myoglobin into casts that can occlude the renal tubules , which blocks urine flow or output .
the other mechanism of renal cellular injury is the peroxidation of lipids in the cellular membrane by the oxygen free radicals produced by iron - containing heme .
rhabdomyolysis releases platelet - activating factor , endothelin , and prostaglandins , which cause vasoconstriction and affect the glomerular filtration rate.4 in this particular case , the cause of resistant , sustained rhabdomyolysis could be multifactorial , related most directly to the muscle necrosis caused by a microthrombotic process secondary to ttp .
rare reports have cited hematological factors in association with or as the cause of rhabdomyolysis.5 in this patient , ttp was diagnosed based on the presence of thrombocytopenia , microangiopathic hemolytic anemia , and central neurological dysfunction .
notably , rhabdomyolysis is implicated as the cause of approximately 5%25% of cases of acute renal failure.6 there are two case reports in the literature worth mentioning .
the first describes a patient with polymyositis who developed ttp , acute renal failure , and rhabdomyolysis after a flare - up of polymyositis following radiation therapy administered for uterine cervical cancer.7 the second case was a patient with thrombotic microangiopathy who presented with fulminant rhabdomyolysis and multiorgan failure .
thrombotic microangiopathy typically presents as fulminating rhabdomyolysis with multiorgan dysfunction.5 although the patient developed multiorgan failure with ttp and severe sepsis , aggressive and continuous interventions including plasma exchange , chemotherapy , antibiotics , and continuous dialysis all contributed to the patient s recovery , despite the lengthy stay in the icu .
conventional treatment for ttp , including plasma exchange , was not effective in isolation . when combined with chemotherapy and steroid therapy , this approach facilitated recovery.8 in conclusion , early , proper treatment of ttp can reduce morbidity and mortality in such patients when administered in combination with the early introduction of interventions such as continuous dialysis , even after the normalization of kidney function .
this very rare or less recognized presentation of a rare disease could contribute to a high mortality rate if not recognized and treated expediently .
finally , it is important to note that the acute renal failure was persistent , possibly because of rhabdomyolysis and not because of ttp alone .
 thrombotic thrombocytopenic purpura ( ttp ) is a rare , life - threatening disorder . 
 this paper describes the case of a 39-year - old sudanese male who presented to the emergency room with fever , jaundice , decreased level of consciousness , and worsening kidney function for 7 days , a high lactate dehydrogenase level ( 1947 ) , severe thrombocytopenia ( platelets 8) , and numerous schistocytes in the peripheral blood smear . 
 the patient was admitted with a diagnosis of ttp for plasma exchange . 
 fourteen days later , his creatinine kinase ( ck ) level rose to > 50,000 iu ; rhabdomyolysis was suggested . 
 continuous venovenous hemodialysis ( cvvhd ) was started . 
 the patient s ck level remained high , despite cvvhd , until the 6th day , after which this parameter gradually started to decrease . 
 this report highlights a resistant case of ttp that presented with concomitant severe rhabdomyolysis , which demanded aggressive , continuous intervention . 
Introduction
Case report
Discussion