Pure red cell aplasia D009

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-9-CM Codes: 284.81
• ICD-10-AM Codes: D60

Brief description

Pure red cell aplasia is a rare cause of severe anaemia.  It is characterised by a very low red cell count and absence or near absence of red cell precursors in the bone marrow, with normal white cell and platelet production.

Confirming the diagnosis

Diagnosis requires a bone marrow examination and a full blood count, with other investigations also likely to be performed to investigate potential causes. 

The relevant medical specialist is a haematologist.

Additional diagnoses covered by SOP

• Red cell aplasia (idiopathic, acquired)

Conditions not covered by SOP

• Agranulocytosis – isolated white cell aplasia^#
• Diamond-Blackfan anaemia^#
• Inherited bone marrow failure syndromes^#
• Myelodysplastic syndrome*
• Paroxysmal nocturnal haemoglobinuria^#
• Thrombocytopaenia – isolated platelet aplasia^#

* another SOP applies

^# non-SOP condition

Clinical onset

The condition tends to occur in older subjects and had an insidious onset.  Clinical onset will be based on the laboratory findings.  Once the diagnosis has been confirmed, the clinical picture may allow backdating of onset to a earlier time. 

Clinical worsening

The only worsening factors are for inability to obtain appropriate clinical management and for being pregnant.  The condition may resolve in some people and persist in others, depending on the underlying cause.  Treatment may include immunosupression and transfusions.   Long term survival is usual with appropriate therapy.