Definition: Pulmonary localization of bone marrow allograft associated-small muscular arteries vasculopathy (pulmonary BMA-SMAV).

Synopsis

 concentric intimal hyperplasia
 concentric medial hyperplasia
 luminal narrowing
 prominent myxoid change
 extravasated red blood cells
 some foamy histiocytes with no evidence of thrombotic microangiopathy

Complications

 digestive ischemia (digestive BMA-SMAV)

• intestinal ischemia (intestinal BMA-SMAV)

 pulmonary BMA-SMAV

• diffuse alveolar damage (DAD)
• pulmonary hemorrhages
  • diffusee alveolar hemorrhages (DAH)

Pathogeny

 The cause of this unusual vasculopathy present in patients after BMT is likely to be multifactorial, involving effects of irradiation, chemotherapy, cyclosporine, and GVHD.

Differential diagnosis

 bone marrow allograft (BMA)

• graft-versus-host disease (GVHD)
• opportunistic infections
• drug toxicity
• thrombotic microangiopathy (TMA)
• veno-occlusive disease (VOID)
• vasculopathy of small muscular arteries (VSMA)
  • digestive VSMA (intestinal VSMA)
  • pulmonary VSMA

See also

 intestinal vasculitis

References

 Selby DM, Rudzki JR, Bayever ES, Chandra RS. Vasculopathy of small muscular arteries in pediatric patients after bone marrow transplantation. Hum Pathol. 1999 Jul;30(7):734-40. PMID: #10414490#