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Bone marrow transplant-associated thrombotic microangiopathy without peripheral
blood schistocytes: a case report and review of the literature
#MMPMID29977661
Wirtschafter E
; VanBeek C
; Linhares Y
Exp Hematol Oncol
2018[]; 7
(?): 14
PMID29977661
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BACKGROUND: Bone marrow transplant-associated thrombotic microangiopathy (TA-TMA)
is a relatively frequent but under-recognized and under-treated hematopoietic
stem cell transplant (HSCT) complication that leads to significant
post-transplant morbidity and mortality. Classic TMA-defining laboratory
abnormalities appear at different times in the course of TA-TMA development, with
schistocytes often appearing later in the disease course. In some severe TMA
cases, schistocytes may be absent due to increased endothelial permeability.
Unfortunately, many clinicians continue to perceive the presence of schistocytes
as an absolute requirement for TA-TMA diagnosis, which leads to delayed
recognition and treatment of this potentially fatal transplant complication.
METHODS: Patient chart review and PubMed literature search using the term,
"transplant-associated thrombotic microangiopathy". CASE PRESENTATION: A
54-year-old male IgG kappa multiple myeloma underwent a reduced intensity
allogeneic HSCT from a 9/10 HLA-matched unrelated donor after conditioning with
fludarabine and melphalan. On day +?27, the patient developed acute kidney injury
followed by repeated episodes of diarrhea and gastrointestinal bleeding
attributed to graft versus host disease (GVHD) and cytomegalovirus (CMV) colitis.
Repeated colonic biopsies suggested CMV infection and GVHD. Despite appropriate
treatment with antiviral therapy and immunosuppressants, the patient's condition
continued to deteriorate. He experienced concomitant anemia and thrombocytopenia
as well as elevated lactate dehydrogenase and low haptoglobin levels, but a
TA-TMA diagnosis was not made due to an absence of schistocytes on peripheral
smear. The patient expired secondary to uncontrolled gastrointestinal bleeding. A
post-mortem analysis of the resection specimen revealed extensive TMA involving
numerous arteries and arterioles in the ileal and colonic submucosa as well as in
the muscularis propria and deep lamina propria of the mucosa. CONCLUSIONS: TA-TMA
can occur in the absence of peripheral blood schistocytes. Our experience
underscores the importance of considering the diagnosis of intestinal TA-TMA in
patients with refractory post-transplant diarrhea and GI bleeding, even if all
classic features are not present.
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