Sjögren's syndrome manifesting as clinicopathological features of TAFRO syndrome: A case report

Abstract

Rationale: TAFRO syndrome is a newly proposed disorder that manifests as thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. In this report, we describe the development of severe TAFRO syndrome-like systemic symptoms during the clinical course of juvenile-onset Sjögren's syndrome in a 32-year-old woman.

Patient concerns: The patient was admitted due to dyspnea, fever, polyarthralgia, and generalized edema. She had been diagnosed with Sjögren's syndrome at the age of 14 years, based on histopathological examination of a biopsy of the minor salivary glands and the development of Raynaud's phenomenon, with no follow-up treatment required. On admission, she presented with anemia, elevated C-reactive protein levels, anasarca, and hepato-splenomegaly. A bone marrow examination revealed increased megakaryocytes with reticulin fibrosis, and the histopathology of an axillary lymph node was consistent with mixed-type Castleman disease. Eventually, she developed thrombocytopenia.

Interventions: Her symptoms fulfilled all of the major and minor categories of the diagnostic criteria for TAFRO syndrome. However, considering her prior diagnosis, we assumed that the clinical presentation was consistent with an acute exacerbation of Sjögren's syndrome. Unlike typical cases of TAFRO syndrome, the administration of relatively low-dose prednisolone relieved her symptoms.

Lessons: Differentiation between TAFRO syndrome and exacerbation of an autoimmune disease is clinically important, although this can be challenging. Identification of specific biomarkers for TAFRO syndrome would be clinically beneficial.

Figure 1

Computed tomography images obtained on admission, showing: (A) bilateral axillary lymphadenopathy (arrow heads), (B) bilateral pleural effusion, (C) hepatosplenomegaly, and (D) massive ascites (arrow heads).

Figure 2

Sections of the bone marrow biopsy, showing: (A) a slightly hypercellular marrow with increased megakaryocytes was observed (hematoxylin and eosin staining, ×100) and (B) reticulin fibrosis (MF-2; reticulin staining, ×100).

Figure 3

Sections of a left axillary lymph node. The architecture of the lymph node was conserved with relatively small follicles. (A) Proliferation of glomerulus-like blood vessels in the germinal center. (B) Proliferation of endothelial venules and infiltration of plasma cells in the interfollicular spaces. The sections that appear in panels (A) and (B) were stained with hematoxylin and eosin. (C) Infiltration of CD38-positive plasma cells into the interfollicular spaces, but not inside the follicles (immunohistochemical staining using an anti-CD38 antibody). Original magnification, ×100.

Figure 4

A section of the minor salivary gland biopsy (hematoxylin and eosin staining; original magnification, ×100), showing infiltration of lymphocytes and plasma cells in the periductal regions, lymphocytic infiltration into the ductal epithelium, and nuclear pseudostratification of the ductal epithelium (Grade 4 sialoadenitis, with a focus score of 6.5).

Figure 5

The patient's clinical course, with the time line of treatments and symptoms, as well as platelet counts (blue line) and serum CRP levels (red line). CRP = C-reactive protein.