CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders
Qinhong Xu1, Jieni Yu1, Xiaoyan Lin2
1Department of Hematology, Zhongshan Hospital, Xiamen University, Fujian Medical University Clinic Teaching Hospital, Xiamen, Fujian, China.
Rationale:
Monoclonal gammopathy of undetermined significance (MGUS) is a clinically asymptomatic clonal plasma cell or lymphoplasmacytic proliferative disorder. Recently, some case reports have described the association of pure red cell aplasia (PRCA) with MGUS, even with a relatively low monoclonal immunoglobulin burden. T large granular lymphocyte leukemia (T-LGLL) is a chronic lymphoproliferative disorder characterized by clonal expansion of T large granular lymphocytes, which is rare in China. There are some reports about T-LGL leukemia in patients with B-cell lymphoma; however, it is very rare that T-LGLL coexists with MGUS and clonal B-cell lymphoproliferative disorders (CB-LPD).
Patient Concerns:
A 77-year-old man was hospitalized because of anemia. He was diagnosed with MGUS, CB-LPD, and PRCA. During the development of the disease, a group of abnormal T lymphocytes was detected by flow cytometry of peripheral blood.
Diagnosis:
Combining clinical manifestations with the result of T cell receptor gene rearrangement and immunophenotype, it was consistent with the diagnosis of T large granular lymphocyte leukemia.
Interventions:
The patient was treat with bortezomib and dexamethasone regimen, Rituximab and sirolimus.
Outcomes:
The patient was transfusion independent after therapies.
Lessons:
We report a patient with 4 concomitant hematological disorders: T-LGLL, MGUS, CB-LPD, and PRCA, aiming to represent the clinical and flow cytometry characteristics of these concomitant diseases, analyze the mechanism between diseases, and provide a clinical reference.
Insights
This case report details a rare instance of four simultaneous blood disorders in one patient: T-LGLL, MGUS, CB-LPD, and PRCA. Treatment led to transfusion independence, offering a clinical reference for these co-occurring conditions.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic plasma cell disorder.
- Pure red cell aplasia (PRCA) and T-large granular lymphocyte leukemia (T-LGLL) are rare hematological conditions.
- Co-occurrence of T-LGLL with MGUS and clonal B-cell lymphoproliferative disorders (CB-LPD) is exceptionally rare.
Observation:
- A 77-year-old male presented with anemia and was diagnosed with MGUS, CB-LPD, and PRCA.
- Flow cytometry revealed abnormal T lymphocytes, leading to a T-LGLL diagnosis.
- The patient exhibited four concomitant hematological disorders.
Findings:
- Diagnosis was confirmed through clinical presentation, T cell receptor gene rearrangement, and immunophenotype.
- Treatment involved bortezomib, dexamethasone, Rituximab, and sirolimus.
- The patient achieved transfusion independence post-therapy.
Implications:
- This case highlights the clinical and flow cytometry characteristics of coexisting T-LGLL, MGUS, CB-LPD, and PRCA.
- Understanding the mechanisms underlying these combined disorders is crucial.
- This report serves as a valuable clinical reference for managing similar complex hematological cases.
