OBJECTIVE: To explore the clinical manifestations, diagnosis, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN). METHODS: The clinical features, bone marrow morphology and immunophenotyping, treatment and prognosis of 4 patients with BPDCN were analyzed retrospectively. RESULTS: 4 patients had bone marrow， spleen and lymph nodes involvement， 2 patients had skin lesions， and 3 patients had central nervous system infiltration． Tailing phenomenon of abnormally cells could be seen in bone marrow. The immunophenotyping showed that CD56, CD4 and CD123 expression was observed in 4 patients， and CD304 in 3 patients． One patient refused chemotherapy and died early. Both patients achieved complete remission after the initial treatment with DA+VP regimen, 1 of them achieved complete remission after recurrence by using the same regimen again． One patient failed to respond to reduced dose of DA+VP chemotherapy, and then achieved complete remission with venetoclax+azacitidine. CONCLUSION The malignant cells in BPDCN patients often infiltrate bone marrow, spleen and lymph nodes, and have specical phenotypes, with poor prognosis. The treatment should take into account both myeloid and lymphatic systems. The treatment containing new drugs such as BCL-2 inhibitors combined with demethylation drugs is worth trying.
Humans ; Dendritic Cells ; Retrospective Studies ; Skin Neoplasms/pathology* ; Antineoplastic Agents/therapeutic use* ; Bone Marrow/pathology* ; Myeloproliferative Disorders ; Hematologic Neoplasms/drug therapy*

OBJECTIVES: To investigate the effectiveness of high-dose chemotherapy combined with autologous hematopoietic stem cell transplantation (ASCT) in the treatment of children with high-risk neuroblastoma (NB). METHODS: A retrospective analysis was performed on 29 children with high-risk NB who were admitted to Shanghai Children's Hospital and were treated with high-dose chemotherapy combined with ASCT from January 2013 to December 2021, and their clinical features and prognosis were analyzed. RESULTS: Among the 29 children treated by high-dose chemotherapy combined with ASCT, there were 18 boys (62%) and 11 girls (38%), with a median age of onset of 36 (27, 59) months. According to the International Neuroblastoma Staging System, 6 children (21%) had stage III NB and 23 children (79%) had stage IV NB, and the common metastatic sites at initial diagnosis were bone in 22 children (76%), bone marrow in 21 children (72%), and intracalvarium in 4 children (14%). All 29 children achieved reconstruction of hematopoietic function after ASCT. After being followed up for a median time of 25 (17, 45) months, 21 children (72%) had continuous complete remission and 8 (28%) experienced recurrence. The 3-year overall survival rate and event-free survival rate were 68.9%±16.1% and 61.4%±14.4%, respectively. Presence of bone marrow metastasis, neuron-specific enolase ≥370 ng/mL and positive bone marrow immunophenotyping might reduce the 3-year event-free survival rate (P<0.05). CONCLUSIONS Children with high-risk NB who have bone marrow metastasis at initial diagnosis tend to have a poor prognosis. ASCT combined with high-dose chemotherapy can effectively improve the prognosis of children with NB with a favorable safety profile.
Child, Preschool ; Female ; Humans ; Male ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Bone Marrow Neoplasms/drug therapy* ; China ; Combined Modality Therapy ; Disease-Free Survival ; Hematopoietic Stem Cell Transplantation ; Neuroblastoma/pathology* ; Prognosis ; Retrospective Studies ; Stem Cell Transplantation ; Transplantation, Autologous

OBJECTIVE: To observe the prevention effect of transcutaneous electrical acupoint stimulation (TEAS) for chemotherapy-related myelosuppression in non-small cell lung cancer. METHODS: A total of 102 patients with non-small cell lung cancer who received initial chemotherapy were randomly divided into a conventional group, a medication group and a TEAS group, 34 cases in each one. The conventional group was treated with chemotherapy of gemcitabine combined with cisplatin and given routine care. On the basis of conventional group's treatment, the medication group was given tablets before chemotherapy, 2-3 tablets each time, 3 times a day. In the TEAS group, on the basis of conventional group's treatment, TEAS was applied at Dazhui (GV 14), Geshu (BL 17), Hegu (LI 4), Zusanli (ST 36) and Sanyinjiao (SP 6) on day 1, 2, 3, 5, 8, 14, 21 and 28 of chemotherapy. The treatment was given 30 min each time and once a day. In the three groups, the treatment for 28 days was as one course and one course of treatment was required. The changes of leukocytes, platelets, erythrocyte, hemoglobin indexes in patients of the three groups were observed one day before chemotherapy and on day 5, 8, 11, 14, 21 and 28 of chemotherapy. The comfort situation of patients was observed one day before chemotherapy and on the 5th, 11th and 21st day of chemotherapy. RESULTS: Compared with before chemotherapy, the leukocyte counts of three groups were decreased at various time points after chemotherapy (<0.05). Compared with the conventional group, the leukocyte counts were higher on day 8 and 14 in the TEAS group and on day 14 in the medication group (<0.05). Compared with before chemotherapy, the platelet count decreased on the day 5, 8, 11 and 14 of chemotherapy in the conventional group (<0.05), and the platelet counts all decreased at each time point after chemotherapy in the medication group (<0.05). The platelet counts of the TEAS group on day 5, 8, 11 and 14 of chemotherapy were higher than those of the conventional group (<0.05), and the platelet counts of the TEAS group on day 5, 8, 11 and 21 of chemotherapy were higher than those of the medication group (<0.05). Compared with the conventional group, the comfort situation scores of the TEAS group were higher on the 5th and 11th days of chemotherapy (<0.05). CONCLUSION Transcutaneous electrical acupoint stimulation can prevent chemotherapy-induced myelosuppression (leukocyte, platelets) in patients with non-small cell lung cancer and improve patient comfort situation.
Acupuncture Points ; Bone Marrow ; drug effects ; Carcinoma, Non-Small-Cell Lung ; drug therapy ; therapy ; Cisplatin ; therapeutic use ; Deoxycytidine ; analogs & derivatives ; therapeutic use ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Lung Neoplasms ; drug therapy ; therapy ; Transcutaneous Electric Nerve Stimulation

therapy-related myelodysplastic syndrome (t-MDS) in breast cancer patients exposed to chemotherapy and/or radiotherapy is significantly high compared to that in other cancer patients. This report reviews the use of hypomethylating agents (HMAs) to treat a 57-year-old woman newly diagnosed with MDS during palliative chemotherapy for metastatic breast cancer. Over a period of 6 years, the patient received several DNA-damaging chemotherapeutics including doxorubicin, cyclophosphamide, and paclitaxel. Repeated thrombocytopenia was the main reason for suspecting secondary hematologic malignancy. She was diagnosed with t-MDS based on bone marrow examination and her treatment history for breast cancer. While azacitidine was originally administered to stabilize MDS, it also stabilized the patient's lung and lymph node metastases without any major toxicity. Therefore, the current case highlights the promising effects of HMAs for treating t-MDS following heavily pretreated breast cancer.]]>
Azacitidine ; Bone Marrow Examination ; Breast Neoplasms ; Breast ; Cyclophosphamide ; DNA Methylation ; Doxorubicin ; Drug Therapy ; Female ; Hematologic Neoplasms ; Humans ; Lung ; Lymph Nodes ; Middle Aged ; Myelodysplastic Syndromes ; Neoplasm Metastasis ; Paclitaxel ; Radiotherapy ; Thrombocytopenia

This article reviewed new trends and controversial issues, including the intensification of chemotherapy and recent brachytherapy (BT) advances, and also reviewed recent consensuses from different societies on the management of locally advanced cervical cancer (LACC). Intensive chemotherapy during and after radiation therapy (RT) was not recommended as a standard treatment due to severe toxicities reported by several studies. The use of positron emission tomography-computed tomography (PET-CT) and magnetic resonance imaging (MRI) for pelvic RT planning has increased the clinical utilization of intensity-modulated radiation therapy (IMRT) for the evaluation of pelvic lymph node metastasis and pelvic bone marrow. Recent RT techniques for LACC patients mainly aim to minimize toxicities by sparing the normal bladder and rectum tissues and shortening the overall treatment time by administering a simultaneous integrated boost for metastatic pelvic lymph node in pelvic IMRT followed by MRI-based image guided adaptive BT.
Bone Marrow ; Brachytherapy ; Consensus ; Drug Therapy ; Electrons ; Humans ; Lymph Nodes ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Pelvic Bones ; Radiotherapy, Intensity-Modulated ; Rectum ; Urinary Bladder ; Uterine Cervical Neoplasms

Although the survival rate of hematologic malignancies in young patients is very high, cytotoxic therapies such as chemotherapy and total body irradiation therapy can significantly reduce a patient's reproductive capacity and cause irreversible infertility. Early ovarian failure also commonly occurs following additional cancer treatment, bone marrow transplantation, or autologous transplantation. Because the risk of early ovarian failure depends on the patient's circumstances, patients with a hematologic malignancy must consult health professionals regarding fertility preservation before undergoing treatments that can potentially damage their ovaries. While it is widely known that early menopause commonly occurs following breast cancer treatment, there is a lack of reliable study results regarding fertility preservation during hematologic malignancy treatment. Therefore, an in-depth discussion between patients and health professionals about the pros and cons of the various options for fertility preservation is necessary. In this study, we review germ cell toxicity, which occurs during the treatment of hematologic malignancies, and propose guidelines for fertility preservation in younger patients with hematologic malignancies.
Autografts ; Bone Marrow Transplantation ; Breast Neoplasms ; Drug Therapy ; Female ; Fertility Preservation* ; Fertility* ; Germ Cells ; Health Occupations ; Hematologic Neoplasms* ; Humans ; Infertility ; Menopause ; Ovary ; Survival Rate ; Transplantation, Autologous ; Whole-Body Irradiation

BACKGROUND/AIMS: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that typically presents in the form of skin manifestations with or without lymph node and bone marrow involvement. Given its rarity and recent recognition as a distinct pathological entity, no standard of treatment exists for this aggressive disease and its prognosis is particularly dismal. METHODS: We retrospectively analyzed clinical features and treatment outcomes of patients who were diagnosed with BPDCN between 2000 and 2014. RESULTS: Ten patients had a median age at diagnosis of 41 years (range, 18 to 79), and seven patients were male. Sites of disease involvement were the skin (n = 7), lymph node (n = 5), bone marrow (n = 2), liver (n = 2), spleen (n = 2), and soft tissue (n = 1). Intensified chemotherapy regimens such as hyperCVAD regimen (cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, cytarabine), and VPDL (vincristine, methylprednisolone, daunorubicin, L-asparaginase) were used as a first-line treatment. Although all patients treated with intensified chemotherapy showed an objective response (five patients with complete response) with median progression-free survival of 11.2 months (range 6.2 to 19.4), complete remission was not sustained for more than 2 years in any case. The response was relatively long-lived compared with previously reported CHOP (doxorubicin, cyclophosphamide, vincristine, prednisone)-like regimens, but the above regimens do not result in long-term remission. CONCLUSIONS: All patients treated with hyperCVAD or VPDL showed an objective response, but the duration of response was relatively short. Thus, the development of more effective induction as well as consolidation treatment strategy should be warranted to improve this rare disease entity.
Bone Marrow ; Cyclophosphamide ; Daunorubicin ; Dendritic Cells* ; Dexamethasone ; Diagnosis ; Disease-Free Survival ; Doxorubicin ; Drug Therapy ; Hematologic Neoplasms ; Humans ; Korea* ; Liver ; Lymph Nodes ; Male ; Methotrexate ; Methylprednisolone ; Prognosis ; Rare Diseases ; Retrospective Studies ; Skin ; Skin Manifestations ; Spleen ; Vincristine

Recent advances in chemotherapy have led to increased survival rates for patients with hematologic malignancies. However, standard chemotherapies, including alkylating agents for non-Hodgkin lymphoma, could induce therapy-related myeloid neoplasms (t-MNs), a group of disorders categorized by the World Health Organization in 2008. Here, we report a case of coexistence of bone marrow (BM)-involved refractory marginal zone B-cell lymphoma (MZL) and therapy-related myelodysplastic syndrome (t-MDS). Simultaneous presence of refractory lymphoma and t-MN in the BM is rare, and this is the first report in Korea. The patient received allogeneic hematopoietic stem cell transplantation (HSCT) to cure both the MZL and t-MDS. Since the HSCT, he has been stable for 21 months without any evidence of recurrence.
Alkylating Agents ; Bone Marrow ; Drug Therapy ; Hematologic Neoplasms ; Hematopoietic Stem Cell Transplantation ; Humans ; Korea ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Lymphoma, Non-Hodgkin ; Myelodysplastic Syndromes* ; Recurrence ; Survival Rate ; World Health Organization

Multiple myeloma is a plasma cell neoplasm mainly involving the bone marrow and skeletal system. Myelomatous pleural effusion is rare, accounting for less than 1%. In cases with high adenosine deaminase (ADA) activity, with lymphocytic exudate in the pleural fluid, tuberculous pleural effusion should be differentiated first. We report herein a rare case of a unilateral pleural effusion in a patient who was undergoing chemotherapy for multiple myeloma, with an ADA level of > 100 IU/L and lymphocytic exudate in the pleural fluid. An acid fast bacillus stain and polymerase chain reaction test for tuberculosis were negative. Consequently, the patient was diagnosed with myelomatous pleural effusion with elevated ADA activity.
Adenosine Deaminase ; Bacillus ; Bone Marrow ; Drug Therapy ; Exudates and Transudates ; Humans ; Multiple Myeloma ; Neoplasms, Plasma Cell ; Pleural Effusion* ; Polymerase Chain Reaction ; Tuberculosis

Here, we report on a 20-year-old patient with a primary nonseminomatous mediastinal germ cell tumor (MGCT) who developed myelodysplastic syndrome (MDS) 2 months following chemotherapy with cisplatin, etoposide, ifosfamide, and paclitaxel. Bone marrow examinations revealed that the MDS was a refractory anemia with excess type II blasts and complex chromosomal abnormalities. With the onset of MDS occurring rapidly following chemotherapy, it is unlikely to have been caused by the therapy. We discuss the association between primary nonseminomatous MGCTs and hematological malignancies, including the possibility of a common clonal origin.
Anemia, Refractory ; Bone Marrow Examination ; Chromosome Aberrations ; Cisplatin ; Drug Therapy* ; Etoposide ; Germ Cells* ; Hematologic Neoplasms ; Humans ; Ifosfamide ; Myelodysplastic Syndromes* ; Neoplasms, Germ Cell and Embryonal* ; Paclitaxel ; Young Adult