What is your preferred strategy for young adults with ITP complicated by recurrent autoimmune neutropenia?
ITP is well controlled on TPO-RA, but AIN which is responsive to steroids recurs despite prior rituximab. Bone marrow biopsy normal and without evidence of large granular lymphocytic leukemia.
Do you consider MMF in addition to TPO-RA versus BTKi?
Answer from: at Community Practice
This is a great question, and I'll say that AIN can be particularly difficult to treat! I'd first ask how low the ANC is and if the patient is presenting with frequent infections/hospitalizations. If not, there may not be a need to treat the AIN (we may just be treating ourselves); oftentimes, there...
Comments
Medical Oncologist at Medical University of South Carolina These are tough cases as we don’t have a cle...
at The Mass General Porphyria Center Agree! Invitae has a comprehensive primary immunod...
Medical Oncologist at Valley Med Onc I heartily agree that it’s important not to ...
I appreciate Dr. @Leaf's answer, especially the point that there might be ample marrow reserve. But I would suggest a different take on this question, namely that if one also has autoimmune neutropenia, this isn't "ITP" anymore, but rather multi-lineage immune cytopenias, or Evans syndrome. My gener...
Dr. Robert Evans of Stanford described an association between autoimmune hemolytic anemia and ITP in 1948, and another paper in 1954 added autoimmune neutropenia to the spectrum of Evans syndrome. I have seen a number of such patients where neutropenia is a predominant symptom. For Evans syndrome, I...
These are tough cases as we don’t have a cle...
Agree! Invitae has a comprehensive primary immunod...
I heartily agree that it’s important not to ...