Development of severe thrombocytopenia with TAFRO syndrome-like features in a patient with rheumatoid arthritis treated with a Janus kinase inhibitor: A case report

Abstract

Rationale: Thrombocytepenia, anasarca, fever, renal insufficiency, and organomegaly (TAFRO) syndrome is a novel disease entity characterized by a constellation of symptoms (thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly). Here, we describe the development of TAFRO syndrome-like features during the treatment of rheumatoid arthritis with a Janus kinase (JAK) inhibitor.

Patient concerns: In this report, a 74-year-old woman treated with a JAK inhibitor (tofacitinib) for rheumatoid arthritis was admitted because of fever and thrombocytopenia.

Diagnoses: On laboratory examination, marked thrombocytopenia and elevated creatinine and C-reactive protein levels were present. A computed tomography scan revealed lymphadenopathy, hepato-splenomegaly, and anasarca. A left axillary lymph node biopsy revealed Castleman's disease-like features. These clinical features satisfied the proposed diagnostic criteria for TAFRO syndrome. Since autoimmune disorders should be excluded when diagnosing TAFRO syndrome, it is not strictly correct to diagnose her as TAFRO syndrome. Therefore, we diagnosed her as rheumatoid arthritis complicated by TAFRO syndrome-like features.

Interventions: The patient was treated with high-dose glucocorticoid, tacrolimus, eltrombopag, intravenous immunoglobulin, and rituximab.

Outcomes: Her condition was refractory to the above-mentioned treatment, and she eventually died because of multi-organ failure 6 months after the first admission.

Lessons: TAFRO syndrome-like features can develop during treatment with a JAK inhibitor for rheumatoid arthritis. Patients with autoimmune diseases complicated by TAFRO syndrome-like features can follow a fatal clinical course, and thus, an intensive combined treatment is warranted for such patients, especially in cases refractory to glucocorticoid.

Figure 1

Computed tomography scan reveals cervical lymphadenopathy (A, arrow), bilateral axillary lymphadenopathy (B, arrow), bilateral pleural effusion, pericardial effusion (C), and hepato-splenomegaly (D).

Figure 2

Histological findings of the axillary lymph node. (A) Atrophic germinal centers and the expansion of the interfollicular zone. Hematoxylin and eosin staining (100 × magnification). (B) Scattered CD38-positive plasma cells in the interfollicular area. Immunohistochemical staining using an anti-CD38 antibody (100 × magnification). (C) Proliferation of small vessels (yellow arrow) are observed in the interfollicular area. Hematoxylin and eosin staining (200 × magnification).

Figure 3

Clinical course of the case.