A Fatal Case of Thrombotic Microangiopathy Without Schistocytosis and Absent Biochemical Markers of Hemolysis.
Biopsy
Erythrocytes, Abnormal
Fatal Outcome
Hemolysis
Hepatomegaly
/ complications
Humans
Inflammation
Integrin beta3
/ metabolism
L-Lactate Dehydrogenase
/ metabolism
Liver
/ pathology
Lung
/ pathology
Male
Microcirculation
Middle Aged
Splenomegaly
/ complications
Stem Cell Transplantation
Thrombocytopenia
/ complications
Thrombosis
/ metabolism
Thrombotic Microangiopathies
/ complications
Transplantation, Autologous
Haptoglobin
LDH
Normal
Schistocyte
Thrombotic microangiopathy
Journal
The American journal of the medical sciences
ISSN: 1538-2990
Titre abrégé: Am J Med Sci
Pays: United States
ID NLM: 0370506
Informations de publication
Date de publication:
05 2020
05 2020
Historique:
received:
24
09
2019
revised:
19
12
2019
accepted:
24
01
2020
pubmed:
9
4
2020
medline:
14
7
2020
entrez:
9
4
2020
Statut:
ppublish
Résumé
Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia with thrombocytopenia. In addition to the primary TMA syndromes, microangiopathic hemolytic anemia with thrombocytopenia can be seen in many systemic diseases. Transplant associated TMA (TA-TMA) affects patients following stem cell or solid organ transplant. A 48-year-old male who underwent autologous stem cell transplant for nonsecretory multiple myeloma was admitted to our hospital with worsening anemia, thrombocytopenia, renal dysfunction and hepatosplenomegaly. Initial blood work revealed rare schistocytes and normal lactate dehydrogenase and haptoglobin levels. He underwent an extensive workup looking for an infectious, inflammatory or malignant etiology but a definitive diagnosis could not be reached. Over his prolonged stay at the hospital, he suffered from multiorgan failure and eventually passed away. An autopsy revealed TMA involving all clinically affected organ systems and was deemed to be the cause of his demise. The absence of typical blood work suggestive of hemolysis does not rule out a diagnosis of TA-TMA. Knowledge of this rare disease entity will help physicians identify and treat this life-threatening condition early and effectively.
Identifiants
pubmed: 32265009
pii: S0002-9629(20)30047-1
doi: 10.1016/j.amjms.2020.01.019
pii:
doi:
Substances chimiques
ITGB3 protein, human
0
Integrin beta3
0
L-Lactate Dehydrogenase
EC 1.1.1.27
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
296-302Informations de copyright
Copyright © 2020 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.