# Schwachman *Diamond Syndrome ICD 10



## emonet01 (Mar 25, 2019)

Does anyone have experience coding Schwachman *Diamond Syndrome. I have come up with at least 3 different codes and I have idea what to go with.

  D61.01    Constitutional (pure) red blood cell aplasia  (Blackfan-Diamond)
  D70.4    Cyclic neutropenia 
  Q45.3    Other congenital malformations of pancreas and pancreatic duct 

Thanks for your help!
Liz


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## Cynthia Hughes (Apr 15, 2019)

*Shwachman Diamond Syndrome*



emonet01 said:


> Does anyone have experience coding Schwachman *Diamond Syndrome. I have come up with at least 3 different codes and I have idea what to go with.
> 
> D61.01    Constitutional (pure) red blood cell aplasia  (Blackfan-Diamond)
> D70.4    Cyclic neutropenia
> ...



Hi Liz,
UPDATE: After responding with information I based on a search with my online coding product, I happened to double-check the alphabetic index file from NCHS and there is an entry for Shwachman's Syndrome (also known as Schwachman's, Shwachman Diamond, and others) directing to - Shwachman's D70.4. Because D70.4 describes only cyclic neutropenia, I would also report codes for other manifestations (eg, malabsorption). Sorry, I didn't see that before my previous response. Cindy

In ICD-9-CM, the listing for Shwachman's Syndrome directed to 288.02 but the syndrome is not listed in ICD-10 alphabetic index. Based on this, it seems you would report only the documented manifestations (eg D70.4 and Q45.3). I could not find anything where Coding Clinic has addressed this condition for ICD-10-CM.

Hope that helps.
Cindy


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