Hemophagocytic lymphohistiocytosis after autologous stem cell transplantation in angioimmunoblastic T-cell lymphoma:A case report

BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL), a unique subtype of peripheral Tcell lymphoma, has relatively poor outcomes. High-dose chemotherapy with autologous stem cell transplantation(ASCT) can achieve complete remission and improve outcomes. Unfortunately, subsequent T-cell lymphoma-triggered hemophagocytic lymphohistiocytosis(HLH) has a worse prognosis than B-cell lymphoma-triggered HLH.CASE SUMMARY We here report a 50-year-old woman with AITL who achieved a favorable outcome after developing HLH 2 mo after receiving high-dose chemotherapy/ASCT. The patient was initially admitted to our hospital because of multiple enlarged lymph nodes. The final pathologic diagnosis, made on biopsy of a left axillary lymph node was AITL(Stage Ⅳ, Group A). Four cycles of the following chemotherapy regimen were administered: Cyclophosphamide 1.3 g, doxorubicin 86 mg, and vincristine 2 mg on day 1;prednisone 100 mg on days 1-5;and lenalidomide 25 mg on days 1-14. The interval between each cycle was 21 d. The patient received a conditioning regimen(busulfan, cyclophosphamide, and etoposide) followed by peripheral blood stem cell infusion. Unfortunately, she developed sustained fever and a low platelet count 17 d after ACST, leading to a diagnosis of HLH after ASCT. During treatment, she experienced thrombocytopenia and Pneumocystis carinii pneumonia. The patient was successfully treated with etoposide and glucocorticoids.CONCLUSION It is possible that development of HLH is related to immune reconstitution after ASCT.

Cyclic adenosine monophosphate involvement in low-dose cyclophosphamide-reversed immune evasion in a mouse lymphoma model

Lymphoma cells mobilize many mechanisms to evade the immune system. There is substantial evidence that CD4＋CD25＋ regulatory T cells （Tregs） play a key role in the control of immune evasion. Tregs can transfer cyclic adenosine monophosphate （cAMP） to effector T cells, suggesting an association between Tregs＇ immune-evasion role and the intracellular cAMP pathway. In this study, we used A20 B-cell lymphoma mice as aggressive tumor models to investigate the mechanism of the depletion of Tregs by low-dose cyclophosphamide （CY, 20 mg/kg）. The tumor-bearing mice had longer survival times and slower tumor growth rates following treatment with CY, but its effects were temporary. Along with the depletion of Tregs by low-dose CY treatment, the expression of interleukin-2 （IL-2） in T effector cells increased, and intracellular cAMP concentrations in immune cells decreased. Our study demonstrates the ability of low-dose CY to reverse Tregs-mediated immune evasion in a mouse model. The changes in intracellular cAMP concentrations correlated with the upregulation of effector T cells and the downregulation of Tregs, indicating the close association of cAMP analogs and low-dose CY in the immune therapy of B-cell lymphoma.

A case of Quebec platelet disorder with interstitial pneumonia

To the Editor:The a activity of fbrinolytic enzymes is complex.Abnormal bleeding can be caused by over-expression of plasminogen activators or a lack of fibrinolysis inhibitor.Quebec platelet disorder(QPD),an autosomal dominant disorder,with increased gene expression of PLAU encoding.urokinase plasminogen activator(u-PA),causes a gain-of-function abnormality in fibrinolysis.Tissue plasminogen activator(t-PA)exists in various tissues and clls.When pulmonary interstitial cells are damaged,t-PA can be released and promote the activation of plasminogen,The interstitial pulmonary fibrosis(IPF)can cause vascular injury and leakage of t-PA,resulting in their elevation in alveoli,Patients can present with fibrinolytic system abnormalities,and anti-fbrinolyt-ic therapy combined with glucocorticoids can effectively prolong the survival time.