Elevated Ristocetin Cofactor, Elevated Adamts13(greater Than 10%25) Elevated D-dimer. Low

Posted on Fri, 6 Sep 2019

Question: Elevated ristocetin cofactor, elevated Adamts13(greater than 10%25) elevated d-dimer. Low red blood cells, normal platelet count. The diagnosis has eluded are physicians. acute episodes similar to delirium. Episode last anywhere from a few hours to a few days. There is a recovery period but he never returns to full function. Meaning there is some decline after each episode and recovery. EEG Revealed possible encephalopathy events. pet scan revealed small uptake in the left frontal lobe. MRI revealed possible asymmetrical sylvian fisher.

Answered by Dr. Shailja Puri (19 hours later)

Brief Answer:
History suggestive of TTP,management is directed towards underlying cause

Detailed Answer:
Hello,
Thanks for posting your query on HealthcareMagic.
The history given by you is suggestive of thrombotic thrombocytopenia purpura (TTP).
TTP is a type of microangiopathic hemolytic anemia 9MAHA) which involves the central nervous system besides low platelet count.
Elevated d-dimer level suggests some thromboembolic phenomenon.
The treatment for TTP is symptomatic and to remove the underlying cause of TTP.
Abnormalities of ADAMTS13 is usually hereditary thus, definitive treatment is not possible.
Management is usually symptomatic and treatment of complications.

Thanks and take care
Dr Shailja Puri

Above answer was peer-reviewed by : Dr. Prasad

Follow up: Dr. Shailja Puri (54 minutes later)

Thank you for the information. it has been a very hard road to get this testing to prove there is some type of clotting issue goung on. I would like to know if aHUS and upshaw syndrome are the same disorder or just very similar?

Answered by Dr. Shailja Puri (13 minutes later)

Brief Answer:
HUS is a type of MAHA, Upshaw syndrome is recessively inherited TTP

Detailed Answer:
Hello and welcome again,

HUS and TTP have microangiopathic hemolytic anemia (MAHA) as a common factor.

However, both have different etiologies, different etiopathogenesis, and different presentations.
HUS primarily affects the kidneys and infection is the major underlying cause.
Upshaw syndrome is a recessively inherited form of TTP.

Upshaw syndrome is clinically similar to TTP.

Thanks and take care
DR Shailja Puri

Above answer was peer-reviewed by : Dr. Arnab Banerjee

Follow up: Dr. Shailja Puri (1 hour later)

thank you for the information. i am asking about Atypical hemolytic uremic syndrome (aHUS) verses upshaw. ahus is a rate Inherited disorder.

Answered by Dr. Shailja Puri (2 hours later)

Brief Answer:
Atypical HUS and Upshaws are hereditary HUS and hereditary Upshaws resp.

Detailed Answer:
Hello and welcome again,

The atypical hemolytic uremic syndrome is a progressive disorder which is caused by a genetic disorder.
Upshaw's is a hereditary type of TTP.

Thanks and take care
Dr Shailja Puri

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Above answer was peer-reviewed by : Dr. Arnab Banerjee