A previously well 54 year-old man presents with confusion. On examination a rash is noted. Temperature 37.1. The initial blood results are provided below.
Venous biochemistry
Na 135
K 3.8
Urea 18 mmol/l -*
Creatinine 177 micromol/l-*
Bilirubin 45 micromol/l -*
Hematology:
Hb 99 g/l
WBC 10.8 x 10(9)/L
Platelet 26 x 10(9)/L-*
Blood film: Schistocytes-*
Coagulation
PT 10 s
APTT 29 s
Fibrinogen 3.0 g/L
What is the most likely diagnosis?
Thrombotic thrombocytopenic purpura
What treatment needs to be instituted urgently?
Plasmapheresis
TTP shows a classic pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, and renal and neurological defects. This is thought to be related to an abnormal metalloproteinase (ADAMST 13). The condition is seen with certain infections, drugs (e.g. calcineurin antagonists, clopidogrel), pregnancy, systemic lupus erythematosus and graft versus host disease.
The labarotary findings in this condition are:
-low platelets
-reduced hemoglobin level with polychromasia, shictocytes and spherocytes
-increased reticulocytes
-reduced haptoglobin and increased lactate dehydrogenase levels
-unconjugated hyperbilirubinemia with urinary urobilinogen
-variable neutrophilia
-increased urea and creatinine levels (greater in hemolytic uremic syndrome)
Venous biochemistry
Na 135
K 3.8
Urea 18 mmol/l -*
Creatinine 177 micromol/l-*
Bilirubin 45 micromol/l -*
Hematology:
Hb 99 g/l
WBC 10.8 x 10(9)/L
Platelet 26 x 10(9)/L-*
Blood film: Schistocytes-*
Coagulation
PT 10 s
APTT 29 s
Fibrinogen 3.0 g/L
What is the most likely diagnosis?
Thrombotic thrombocytopenic purpura
What treatment needs to be instituted urgently?
Plasmapheresis
TTP shows a classic pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, and renal and neurological defects. This is thought to be related to an abnormal metalloproteinase (ADAMST 13). The condition is seen with certain infections, drugs (e.g. calcineurin antagonists, clopidogrel), pregnancy, systemic lupus erythematosus and graft versus host disease.
The labarotary findings in this condition are:
-low platelets
-reduced hemoglobin level with polychromasia, shictocytes and spherocytes
-increased reticulocytes
-reduced haptoglobin and increased lactate dehydrogenase levels
-unconjugated hyperbilirubinemia with urinary urobilinogen
-variable neutrophilia
-increased urea and creatinine levels (greater in hemolytic uremic syndrome)
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