Recurrent anti-GBM disease with T-cell large granular lymphocytic leukemia: A case report

Abstract

Rationale: Anti-glomerular basement membrane disease (anti-GBM disease) is a rare small vessel vasculitis caused by autoantibodies directed against the glomerular and alveolar basement membranes. Anti-GBM disease is usually a monophasic illness and relapse is rare after effective treatment. This article reports a case of coexistence of recurrent anti-GBM disease and T-cell large granular lymphocytic (T-LGL) leukemia.

Patient concerns: A 37-year-old man presented with hematuria, edema, and acute kidney injury for 2 months.

Diagnosis: Anti-GBM disease was diagnosed by renal biopsy, in which crescentic glomerulonephritis was observed with light microscopy, strong linear immunofluorescent staining for immunoglobulin G on the GBM and positive serum anti-GBM antibody. Given this diagnosis, the patient was treated with plasmapheresis, steroids, and cyclophosphamide for 4 months. The anti-GBM antibody titer was maintained to negative level but the patient remained dialysis-dependent. One year later, the patient suffered with a relapse of anti-GBM disease, after an extensive examination, he was further diagnosed T-LGL leukemia by accident.

Interventions: The patient received cyclosporine A therapy for T-LGL leukemia.

Outcomes: After treatment with cyclosporine A, serum anti-GBM antibody became undetectable. During the 16 months follow-up, anti-GBM titer remained normal and abnormal T-lymphocytes in the bone marrow and peripheral blood were also decreased.

Lessons: T-LGL leukemia is an indolent lymphoproliferative disorder that represents a monoclonal expansion of cytotoxic T cells, which has been reported to be accompanied by some autoimmune diseases. This is the first report of coincidence of T-LGL leukemia and anti-GBM disease, and suggests there are some relationships between these 2 diseases. Clinical physicians should exclude hematological tumors when faced with recurrent anti-GBM disease.

Figure 1

The renal pathology of the patient's renal biopsy. (A) The silver staining of the kidney biopsy showed an active cellular crescent (×400); (B) The immunofluorescence showed strong linear glomerular basement membrane staining of IgG. (×400). IgG = immunoglobulin G.

Figure 2

The peripheral blood and bone marrow smear. (A) Peripheral blood smear manifested classic large lymphocytes (arrow) with a condensed round nuclear, abundant pale basophilic cytoplasm, and small azurophilic granules (×1000); (B) Bone marrow smear showed a few macrolymphocytes and atypical lymphocytes (arrow) (×1000).

Figure 3

The results of peripheral blood flow cytometry. (A) CD45/SSC gating showed 40% lymphocytes (P2) and 16% CD3+ CD4− CD8− abnormal T lymphocytes (dark green dots, P3); (B) The abnormal T lymphocytes (dark green dots) were CD3+ CD8−; (C) The abnormal T lymphocytes (dark green dots) were CD3+ CD4−; (B) The abnormal T lymphocytes (dark green dots) were TCRγδ+ and TCRαβ- (P.S.TCR AB = TCRαβ, TCR CD = TCR γδ).

Figure 4

During the 16-mo follow-up, (A) The abnormal T lymphocytes in the peripheral blood and bone marrow were improved after the treatment of 100 mg cyclosporine A q12h, but in 2017.8, the peripheral abnormal T lymphocytes have a rebound and the plasma concentration of cyclosporine A was low so the dose was adjusted to 150 mg q12h and the peripheral abnormal T lymphocytes decreased again; (B) The titer of the anti-GBM antibody returned normal. GBM = glomerular basement membrane.