Archives

  • 2018-07
  • 2019-04
  • 2019-05
  • 2019-06
  • 2019-07
  • 2019-08
  • 2019-09
  • 2019-10
  • 2019-11
  • 2019-12
  • 2020-01
  • 2020-02
  • 2020-03
  • 2020-04
  • 2020-05
  • 2020-06
  • 2020-07
  • 2020-08
  • 2020-09
  • 2020-10
  • 2020-11
  • 2020-12
  • 2021-01
  • 2021-02
  • 2021-03
  • 2021-04
  • 2021-05
  • 2021-06
  • 2021-07
  • 2021-08
  • 2021-09
  • 2021-10
  • 2021-11
  • 2021-12
  • 2022-01
  • 2022-02
  • 2022-03
  • 2022-04
  • 2022-05
  • 2022-06
  • 2022-07
  • 2022-08
  • 2022-09
  • 2022-10
  • 2022-11
  • 2022-12
  • 2023-01
  • 2023-02
  • 2023-03
  • 2023-04
  • 2023-05
  • 2023-06
  • 2023-07
  • 2023-08
  • 2023-09
  • 2023-10
  • 2023-11
  • 2023-12
  • 2024-01
  • 2024-02
  • 2024-03
  • transcription factors Regarding the treatment of HLH with HL

    2019-05-29

    Regarding the treatment of HLH with HLH-1994, the estimated 3-year probability of survival overall was 55%. According to a previous comprehensive review study, ferritin level may provide a prognostic marker, and regular ferritin measurements may be useful in predicting outcomes in HLH patients. Our patient was treated with HLH-1994 protocol, and his ferritin dosage decreased from 59384 ng/ml to 3653 ng/ml. More importantly, his fever and cytopenia improved after the treatment. Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon type of skin lymphoma. The natural history, optimal treatment strategy, and prognostic factors associated with this malignancy are not well-defined. Rein Willemze et al identified one hundred fifty-six patients with SPTCL. Hemophagocytic syndrome (HPS) was a presenting feature in 37% of patients, and >90% of patients required treatment at diagnosis. In our patient, he was finally diagnosed as panniculitis-like T cell lymphoma confirmed by a soft tissue incisional biopsy in which immunohistochemistry stain showed CD3(+), CD20(−), CD56(−), CD4(−), CD8(+), TIA-1(+) nine months after initial diagnosis of HLH. However, according to the recent World Health Organization–European Organization for Research and Treatment of Cancer classification (WHO-EORTC), there has been a redefinition of the diagnostic criteria of subcutaneous panniculitis-like T-cell lymphoma to primary cutaneous T-cell lymphoma expressing α/β T-cell receptor (TCR) phenotype. transcription factors We were limited in our ability to make further differential diagnosis of α/β or γ/δ TCR phenotype of primary cutaneous T-cell lymphoma because there were no available tests for TCR gene rearrangement. Thus, this patient\'s final diagnosis of subcutaneous panniculitis-like T cell lymphoma was based on the pathologic report and clinical presentation. S.R. Hoque et al reported two subsets of SPTCL: those derived from gamma-delta T transcription factors which carry a poor prognosis, and are usually CD56 positive, and a more indolent group derived from alpha-beta T cells. In this study, two patients were CD56 positive, and both developed the hemophagocytic syndrome. Our patient belonged to the CD56 negative. Guenova E et al reported treatment with systemic corticosteroids induced a complete remission in patients with SPTCL. With our patient, he received steroid therapy using the HLH-1994 protocol, which may assist treatment of SPTCL. In addition to steroid therapy, chemotherapy can also be beneficial for SPTCL patients. Zhi Li Hu et al reported treatment with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone can be efficacious for a 12-year-old Chinese boy who was diagnosed with subcutaneous panniculitis-like T-cell lymphoma who presented with multiple indolent erythematous subcutaneous nodules on both extremities. Avninder Singh et al reported a successful regression in skin lesions and constitutional symptoms in a 22-year-old woman diagnosed as SPTCL, who presented with variably sized multiple nodules on both legs after five cycles of a cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) regimen. In addition, Yu et al reported that allogeneic hematopoietic stem cell transplantation could possibly provide survival benefits to T-cell lymphoma-associated HLH by graft-versus-lymphoma effect. In conclusion, the underlying disease leading to HLH is crucial to each patient\'s treatment and outcome. However, not all patients\' underlying disease can be identified when hemophagocytic lymphohistiocytosis is diagnosed. Similar to our patient, subcutaneous panniculitis-like T-cell lymphoma may present initially with hemophagocytic syndrome without evidence of other etiology. While the optimal treatment of HLH remains controversial, current treatment regimes usually involve high dose corticosteroids, etoposide or cyclosporin. The HLH-1994 protocol is considered useful for treatment of hemophagocytic lymphohistiocytosis, but corticosteroid administration is often found to be a risk factor attributed to developing PjP in these patient populations. The threshold of “how much for how long” regarding steroids and risk for PjP is unknown. Thus more specific treatment for underlying disease like SPCTL may be the most important future consideration.