&lt;p&gt;Pathophysiologic Mechanisms And Management Of Large Granular Lymphocytic Leukemia Associated Pure Red Cell Aplasia&lt;/p&gt;

Qiu, Zhiyuan; Qin, Rong; Tian, Guangyu; Wang, Yan; Zhang, Yeqing

doi:10.2147/ott.s222378

Cited by 12 publications

(14 citation statements)

References 98 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The discovery of STAT3 mutations has shed light on the genetic basis of LGLL pathogenesis [ 26 , 46 ]. Although T-LGLL patients with PRCA have been reported to preferentially harbor STAT3 mutations [ 44 , 47 , 48 ], none were found in the PRCA patients in this cohort. This is likely due to the small number of cases investigated.…”

Section: Discussionmentioning

confidence: 70%

“…It is uncertain the mechanism of how LGLL causes cytopenia, but the direct cytotoxicity effect on hematopoietic cells is an appealing hypothesis. In patients with PRCA, both in vitro and in vivo data suggested that erythropoiesis at a level of differentiation between early and late erythroid progenitors was inhibited by large granular lymphocytes, which was mediated by direct cell-cell interaction [ 48 , 50 ]. Immature erythroid elements were essentially absent in PRCA patients but present in non-PRCA patients, suggesting LGLL cells inhibited both immature and mature erythroid elements that might share similar cell surface antigens at successive stages of differentiation.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)

et al. 2022

View full text Add to dashboard Cite

Patients with large granular lymphocytic leukemia (LGLL) frequently present with neutropenia. When present, anemia is usually accompanied by neutropenia and/or thrombocytopenia and isolated anemia is uncommon. We evaluated a cohort of 244 LGLL patients spanning 15 years and herein report the clinicopathologic features of 34 (14%) with isolated anemia. The patients with isolated anemia showed a significantly male predominance (p = 0.001), a lower level of hemoglobulin (p < 0.0001) and higher MCV (p = 0.017) and were less likely to have rheumatoid arthritis (p = 0.023) compared to the remaining 210 patients. Of the 34 LGLL patients with isolated anemia, 13 (38%) presented with pure red cell aplasia (PRCA), markedly decreased reticulocyte count and erythroid precursors, and more transfusion-dependence when compared to non-PRCA patients. There was no other significant clinicopathologic difference between PRCA and non-PRCA patients. 32 patients were followed for a median duration of 51 months (6–199). 24 patients were treated (11/11 PRCA and 13/21 non-PRCA patients, p < 0.02). The overall response rate to first-line therapy was 83% [8/11 (72.7%) for PRCA, 12/13 (92.3%) for non-PRCA], including 14 showing complete response and 6 showing partial response with a median response duration of 48 months (12–129). Half of non-PRCA patients who were observed experienced progressive anemia. During follow-up, no patients developed neutropenia; however, 5/27 (18.5%) patients developed thrombocytopenia. No significant difference in overall survival was noted between PRCA and non-PRCA patients. In summary, this study demonstrates the unique features of LGLL with isolated anemia and underscores the importance of recognizing LGLL as a potential cause of isolated anemia, which may benefit from disease-specific treatment. LGLL patients with PRCA were more likely to require treatment but demonstrated similar clinicopathologic features, therapeutic responses, and overall survival compared to isolated anemia without PRCA, suggesting PRCA and non-PRCA of T-LGLL belong to a common disease spectrum.

show abstract

Section: Discussionmentioning

confidence: 70%

Section: Discussionmentioning

confidence: 99%

Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)

et al. 2022

View full text Add to dashboard Cite

show abstract

“…This may not be a coincidence, as APS-1, PRCA and T-LGLL are all rare diseases, and PRCA and LGLL can even develop in siblings with the same AIRE mutation [ 18 ]. To date, some mechanisms for patients diagnosed with LGLL with PRCA have been explored [ 19 ]. Regarding molecular mechanisms, STAT3 mutations have been found in some PRCA patients with LGLL [ 20 ], though it remains uncertain whether STAT3-mutated T cells have an inhibitory effect on erythroid cell production.…”

Section: Discussionmentioning

confidence: 99%

Acquired pure red cell aplasia and T cell large granular lymphocytic leukaemia in patients with autoimmune polyglandular syndrome type 1

et al. 2021

View full text Add to dashboard Cite

Background Pure red cell aplasia (PRCA) and large granular lymphocytic leukaemia (LGLL) are very rare complications of autoimmune polyendocrine syndrome type 1 (APS1). Here, we report a case of APS1 with PRCA and LGLL. Previous cases were reviewed, and possible mechanisms are discussed. Case presentation A 31-year-old female presented with anaemia and was diagnosed with PRCA in our centre. She also had hypoparathyroidism for 24 years, premature ovarian failure for 10 years, osteoporosis for 5 years, recurrent pneumonia with bronchiectasis for 4 years and chronic diarrhoea for 1 year. Boosted whole-exome analysis showed AIRE heterozygous mutations, confirming the diagnosis as APS1. LGLL was diagnosed during follow-up. The PRCA responded well to glucocorticoid. treatment Conclusion AIRE is causally related to the development of LGLL and consequent PRCA, which may be due to some immunological mechanisms.

show abstract

“…One possible mechanism is that CTX reduces the count of cytotoxic T lymphocytes (CTLs) that damage antibody-bound erythroblasts directly. Another possible mechanism is that CTX reduces the cytotoxic T lymphocytes-mediated damage to hematopoietic progenitors, which leads to anemia[ 13 ]. In our case, combined chemotherapy, splenectomy, and other treatments that pose a greater risk of side effects were avoided.…”

Section: Discussionmentioning

confidence: 99%

Treatment for CD57-negative γδ T-cell large granular lymphocytic leukemia with pure red cell aplasia: A case report

Xiao¹,

Chen²,

Dong³

et al. 2021

WJCC

View full text Add to dashboard Cite

BACKGROUND T-cell large granular lymphocytic leukemia (T-LGLL) is a rare type of aplastic anemia with diverse clinical manifestations. Concomitant diseases are often present at the first manifestation. We describe the treatment of a patient with CD57-negative γδT-LGLL with pure red cell aplasia (PRCA). CASE SUMMARY A 34-year-old woman with a 20-year history of anemia visited our hospital owing to severe dizziness and was admitted. Her condition was diagnosed as CD57-negative γδT-LGLL with PRCA through bone marrow cytology, bone marrow pathology, bone marrow flow cytometry, bone marrow multiplex polymerase chain reaction combined with fluorescent fragment analysis, and other tests. Treatment with prednisone, methotrexate, and subcutaneous erythropoietin did not significantly change her hemoglobin level. After treatment with oral cyclophosphamide for 3 mo, her hemoglobin level increased to approximately 100 g/L. After 5 mo of treatment, the patient could perform activities of daily living independently. CONCLUSION The treatment of CD57-negative γδT-LGLL with PRCA with cyclophosphamide helps to improve prognosis.

show abstract

<p>Pathophysiologic Mechanisms And Management Of Large Granular Lymphocytic Leukemia Associated Pure Red Cell Aplasia</p>

Cited by 12 publications

References 98 publications

Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)

Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)

Acquired pure red cell aplasia and T cell large granular lymphocytic leukaemia in patients with autoimmune polyglandular syndrome type 1

Treatment for CD57-negative γδ T-cell large granular lymphocytic leukemia with pure red cell aplasia: A case report

Contact Info

Product

Resources

About