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Case Reports
. 2020 Oct 13:21:e926721.
doi: 10.12659/AJCR.926721.

An Autopsy Case of TAFRO Syndrome with Type II Respiratory Failure

Mikio Wada  1 , Akihiro Nagata  2 , Atsushi Kawashima  3 , Keizo Kagawa  3
Affiliations
Case Reports

An Autopsy Case of TAFRO Syndrome with Type II Respiratory Failure

Mikio Wada et al. Am J Case Rep. .

Abstract

BACKGROUND TAFRO syndrome (thrombocytopenia, anasarca, fever, myelofibrosis, renal dysfunction, and organomegaly) is a systemic inflammatory disorder. The histological features of TAFRO syndrome are not fully understood and few autopsy cases have been reported. CASE REPORT A 66-year-old man with type II respiratory failure was diagnosed with TAFRO syndrome. He was initially treated with tocilizumab. Although some improvements were observed, his condition worsened, and the medication was switched to rituximab. His condition remained steady for 1 year with intermittent artificial ventilation. However, he died due to exacerbation of respiratory failure about 20 months after diagnosis. An autopsy revealed mucous fluid retention in the spaces between the axis cylinder and the myelin sheath of peripheral nerves and among the peripheral nerves, suggesting that this retention contributed to neurodegeneration with demyelination. Skeletal muscles, including respiratory muscles, were highly atrophic, which could have led to type II respiratory failure. CONCLUSIONS Fluid accumulation other than pleural effusion and ascites could occur in intra-organs at a cellular level.

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Conflict of interest statement

Conflict of interest: None declared

Conflicts of interest

None.

Figures

Figure 1.
Figure 1.
Histological findings on biopsy (our previous study [5]). (A) Histological appearance of left axillary lymph node with hematoxylin and eosin stain. Many lymphoid follicles with unclear atrophic germinal centers and expansion of interfollicular zone were apparent. Original magnification ×100. (B) A peripheral mantle layer was developed with a concentric cellular distribution. Original magnification ×200. (D) Arborized blood vessels were present and we noted infiltration of small lymphocytes and plasma cells in the interfollicular zone. Original magnification ×400. (C) Increase in megakaryocytes of the bone marrow was evident by hematoxylin and eosin stain. Original magnification ×200. (D) Silver impregnation stain confirmed an increase in reticular fibers. Original magnification ×400.
Figure 2.
Figure 2.
Clinical course.
Figure 3.
Figure 3.
Histological findings on autopsy. (A) Alcian Blue staining and (B) hyaluronidase staining of peripheral nerves. Spaces that were positive for Alcian Blue staining (arrows) and negative for hyaluronidase staining (arrow head) were observed around the peripheral nerves. Retention of mucus was observed around and among the peripheral nerves of the peripheral nerves. * Axis cylinder. + myelin sheath. Original magnification ×20. (C, D) Klüver-Barrera staining of proximal peripheral nerves revealed demyelination. Original magnification ×20 for (C) and ×10 for (D). (E) Hematoxylin and eosin staining of respiratory muscles. Highly atrophic changes were observed. Original magnification ×10.
Figure 4.
Figure 4.
(A) Hematoxylin and eosin staining of lymph nodes (original magnification ×4) and (B) CD34 immunostaining of lymph nodes (original magnification ×10). Overall, lymph nodes were atrophic, and high endothelial venules were relatively notable. (C, D) Renal histological findings include a membranoproliferative glomerulonephritis-like appearance. (C) Hematoxylin and eosin staining (original magnification ×10) and (D) periodic acid methenamine silver staining (original magnification ×20). Almost all glomeruli showed lobular formation with mesangial proliferation. Many large glomeruli showed crescent formation.
See this image and copyright information in PMC

References

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    1. Aoki T, Wada M, Kawashima A, et al. Tocilizumab-resistant TAFRO syndrome complicated by type II respiratory failure. Intern Med. 2017;56:3249–54. - PMC - PubMed

Publication types

Supplementary concepts