Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia affecting children 2-5 years old. The clinical presentation ranges from self-resolving localized disease to fulminant, fatal disseminated disease. While the most common presentation of LCH are small, translucent crusted papules on the trunk, intertriginous areas, and scalp, it may present as crusted plaques and alopecia. A 3-year-old male presented with a 4-month history of solitary, well-defined, hyperpigmented plaque with yellow-brown crust on the left parieto-occipital area of the scalp measuring 1.5 x 1.5 cm and a solitary, well-defined, hairless patch with areas of erythema on the left parieto-occipital area measuring 5.0 x 6.0 cm. Scalp biopsy revealed diffuse collection of lymphohistiocytes interspersed with distinct kidney bean-shaped cells. CD1a is positive for cells of interest. Skeletal survey revealed lytic lesions involving the skull, thoracic cage, spine, pelvis, and upper and lower extremities. The rest of the physical examination findings revealed lymphadenopathy, crackles, globular abdomen with right and left upper quadrant dullness. The patient had episodes of fever, difficulty of breathing, and abdominal pain. The patient received chemotherapy as multisystem LCH based on prednisone and vinblastine. Following 3 courses of chemotherapy, there is noted hair regrowth and sloughing off of crust.

Human ; Male ; Child Preschool: 2-5 Yrs Old ; Alopecia ; Histiocytosis, Langerhans-cell ; Vinblastine

Antibodies, Monoclonal, Humanized/adverse effects* ; Hodgkin Disease/drug therapy* ; Humans ; Vinblastine

Objective: To investigate the pathological characteristics and clinical prognosis of nodular sclerosis grade 2 of classic Hodgkin's lymphoma (cHL-NS2) in our cancer center. Methods: A retrospective collection of 23 cases of cHL-NS2 admitted in Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from July 2008 to April 2019 was performed. Fifty-five cases of nodular sclerosis grade 1 of classical Hodgkin's lymphoma (cHL-NS1) during the same period were selected as control group. Survival curves were plotted using the Kaplan-Meier method, and Cox regression model was used to analyze the influencing factors for survival. Results: The median age of 23 cases of cHL-NS2 was 30 years old. Five cases had extra nodal invasion, and 19 cases were Ⅰ-Ⅱ stage based on Ann Arbor system. The pathological morphology of cHL-NS2 showed that the lymph node structure was completely destroyed and was divided into nodules by thick collagen. The tumor cells in the nodules were abundant and proliferated in sheets. The boundaries between the tumor cells were not clear. The incidence of tumor necrosis in cHL-NS2 was 43.5% (10/23), which was significantly higher than 18.2% (10/55) in cHL-NS1 (P=0.040). The 3-year progression-free survival (PFS) rate of patients in the cHL-NS2 group was 58.1%, which was significantly lower than 89.7% in the cHL-NS1 group (P=0.002). In all of 78 cases, the 3-year PFS rate of patients who did not obtain complete response (CR) was 67.1%, which was significantly lower than 92.2% in patients who achieved CR (P=0.030). Multivariate Cox regression analysis demonstrated that both cHL-NS2 and failure to obtain CR by first-line treatment were independent indicators for short PFS time (P<0.05). Conclusions: In cHL-NS2, the morphology of tumor cells are diverse, and tumor necrosis can be easily found. Under the current first-line treatments of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) or bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone (BEACOPP), cHL-NS2 is an independent indicator for worse PFS.
Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Bleomycin/therapeutic use* ; Cyclophosphamide/therapeutic use* ; Dacarbazine/therapeutic use* ; Doxorubicin/therapeutic use* ; Etoposide/therapeutic use* ; Hodgkin Disease/drug therapy* ; Humans ; Necrosis/drug therapy* ; Prednisone/therapeutic use* ; Prognosis ; Retrospective Studies ; Sclerosis/drug therapy* ; Vinblastine/therapeutic use* ; Vincristine/therapeutic use*

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.
Airway Obstruction ; Biopsy ; Child ; Cladribine ; Cytarabine ; Drug Therapy ; Dyspnea ; Emergencies ; Estrogens, Conjugated (USP) ; Female ; Histiocytosis, Sinus ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Methotrexate ; Neck ; Prednisone ; Radiotherapy ; Remission, Spontaneous ; Trachea ; Vinblastine ; Vincristine

A 40-year-old man presented with pruritic, multiple, variable-sized, erythematous umbilicated papules on the trunk and both extremities for 4 months. He was diagnosed with Hodgkin's lymphoma (stage IIA) after histopathologic examination of a neck mass that developed a month ago. A punch biopsy was performed on his right lower leg. Histological examination showed transepidermal elimination of the degenerated collagen. Masson's trichrome staining was performed to distinguish collagen fibers from the muscular tissue; using Masson's stain, the collagen appeared as a bluish color crossing from the dermis to the epidermis. The diagnosis of acquired reactive perforating collagenosis was made. The skin lesions showed much improvement after 6 cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine chemotherapy. Acquired perforating disorders are a group of cutaneous disorders that occur in adults with chronic kidney disease or diabetes mellitus. Cases of acquired perforating disorders associated with Hodgkin's lymphoma have been rarely reported in the English literature. To our knowledge, this is the first case of acquired reactive perforating collagenosis in a Korean patient with Hodgkin's lymphoma.
Adult ; Biopsy ; Bleomycin ; Collagen ; Dacarbazine ; Dermis ; Diabetes Mellitus ; Diagnosis ; Doxorubicin ; Drug Therapy ; Epidermis ; Extremities ; Hodgkin Disease ; Humans ; Leg ; Neck ; Renal Insufficiency, Chronic ; Skin ; Vinblastine

To investigate the clinical efficacy and toxicities for the NAPD regimen (vinorelbine, cytarabine, cisplatin, and dexamethasone) in the treatment of recurrent refractory diffuse large B-cell lymphoma.
 Methods: A total of 30 patients identified with recurrent refractory diffuse large B-cell lymphoma were enrolled in this retrospective study. The curative efficacy of NAPD regimen was evaluated after 2 consecutive cycles. The toxicities and adverse reaction were evaluated after 1 cycle. The objective response rate (ORR), overall survival (OS), progress free survival (PFS), and the rates of 1, 2, and 4-year OS and PFS were analyzed. The prognosis was evaluated with univariate analysis.
 Results: The ORR was 56.7% and clinical benefit rate (CBR) was 83.3% after 2 cycles. Five patients achieved complete remission, 12 achieved partial remission, and 8 achieved stable disease. The median OS was 22 (1.5-140) months. The 1, 2, and 4-year OS rates were 59.1%, 48.2%, and 40.2%, respectively. The median PFS was 14 (1.5-140) months. The 1, 2 and 4-year PFS rates were 56.3%, 42.2%, and 31.7%, respectively. The main adverse reaction was myelosuppression. Three patients suffered from grade III-IV leukopenia and 1 thrombocytopenia. Grade I-II gastrointestinal toxicity was 20%. No heart, liver, and kidney damages at grade III-IV were observed.
 Conclusion: The NAPD regimen is effective and its toxicity is well tolerated for the treatment of recurrent refractory diffuse large B-cell lymphoma. It is a salvage chemotherapy regimen worth to be verified.
Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Cisplatin ; administration & dosage ; Cytarabine ; administration & dosage ; Dexamethasone ; administration & dosage ; Humans ; Induction Chemotherapy ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; mortality ; Neoplasm Recurrence, Local ; drug therapy ; mortality ; Retrospective Studies ; Salvage Therapy ; methods ; Treatment Outcome ; Vinblastine ; administration & dosage ; analogs & derivatives ; Vinorelbine

Erdheim-Chester disease (ECD) is a rare non-Langerhan's cell histiocytosis disorder characterized by replacement of normal tissue by lipid-laden histiocytes affecting various organs. A few pediatric cases have been reported worldwide. Here we present a child with leukemia who was diagnosed as ECD. A 2-year and 11-month old boy diagnosed with high risk acute lymphoblastic leukemia (ALL) at the age of 17 months, received allogeneic hematopoietic stem cell transplantation (HSCT) at the age of 2 years old. Six months after the transplantation, the patient was admitted to the hospital with palpable left calf nodules. Bone marrow study suggested ECD without leukemia with complete chimerism status. Excisional biopsy of the left calf nodule showed ‘aggregation of non-Langerhan's cell type epitheloid histiocytes’; clinically suggestive of ECD. The patient was started on vinblastine and corticosteroid treatment.
Biopsy ; Bone Marrow ; Child* ; Chimerism ; Erdheim-Chester Disease* ; Hematopoietic Stem Cell Transplantation ; Histiocytes ; Histiocytosis ; Humans ; Leukemia* ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Vinblastine

A 22-year-old woman was diagnosed with intermediate risk stage II Hodgkin lymphoma and treated with three cycles of adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD) followed by involved-field radiation therapy. A complete metabolic remission was achieved after two cycles of ABVD, which was maintained until three years after completion of treatment. Follow-up FDG-PET/CT four years after completion of treatment, however, showed a new FDG-avid (Deauville score of 4) lesion in the right scapula, suggesting relapsed disease. Computer tomography (CT)-guided biopsy of this lesion was performed and subsequent histological examination revealed a radiation-induced giant cell granuloma.
Biopsy ; Bleomycin ; Dacarbazine ; Doxorubicin ; Female ; Follow-Up Studies ; Giant Cells ; Granuloma, Giant Cell ; Hodgkin Disease ; Humans ; Scapula ; Vinblastine ; Young Adult

OBJECTIVETo characterize the clinical features of desmoid tumor, assess the efficacy of conservative chemotherapy for inoperable desmoid tumor and analyze the prognostic factors.

METHODSFrom August 2009 to December 2013, 52 patients with inoperable desmoid tumor were treated in our department and received chemotherapy with vinorelbine combined with low-dose methotrexate. The clinical data of the patients were analyzed retrospectively.

RESULTSThe patients studied included 22 male and 30 female patients with the age of disease onset ranging from 2 to 46 years (mean 18.7 years). The lesions occurred most frequently in the lower limbs (36.5%, 19/52) and the tumor size ranged from 2.7 to 37 cm (mean 9.5 cm). The patients were followed up for a median of 29 months (7 to 64 months). The chemotherapy lasted for 4 to 30 months (median 12 months). After completion of the chemotherapy, 1 patient had a complete response (CR), 18 showed partial responses (PR), 27 cases had stable disease (SD), and 6 had progressive disease (PD), with an overall response rate (ORR) of 88.5%. The progression-free survival (PFS) time of the patients ranged from 4 to 63 months (median 26.5 months) with a 2-year PFS rate of 76.7% and 5-year PFS rate of 41.9%. A longer chemotherapy duration (over 12 months) was associated with a more favorable prognosis. No significant differences in PFS were found between the patients stratified by gender, age of disease onset, age when receiving chemotherapy, tumor site, or tumor size.

CONCLUSIONFor recurrent, inoperable and progressive desmoid tumor, long enough cycles of vinorelbine combined with low-dose methotrexate can be an effective and safe option for tumor control.

Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; Child ; Child, Preschool ; Disease-Free Survival ; Female ; Fibromatosis, Aggressive ; drug therapy ; Humans ; Male ; Methotrexate ; administration & dosage ; therapeutic use ; Middle Aged ; Prognosis ; Retrospective Studies ; Vinblastine ; administration & dosage ; analogs & derivatives ; therapeutic use ; Young Adult

BACKGROUNDThe aim of this study was to assess the efficacy and safety of vinorelbine and cisplatin (NP chemotherapy) alone or in combination with Aidi injection for the treatment of advanced nonsmall cell lung cancer (NSCLC).

METHODSPertinent publications were identified in PubMed, EMBASE, Cochrane Library, CNKI, CQVIP, and Wanfang databases, up to December 8, 2015. After quality assessment of all included randomized controlled trials evaluating Aidi injection combined with NP chemotherapy for the treatment of advanced NSCLC, a meta-analysis was performed by Review Manager 5.2 and STATA 12.0 for statistical analyses.

RESULTSTwelve studies including 509 and 503 cases in the experimental and control groups, respectively, were finally analyzed. The meta-analysis revealed that when cisplatin dose ranging from 20 to 40 mg/m 2 , combination of Aidi injection and NP chemotherapy was statistically different compared with NP chemotherapy alone in enhancing efficiency (relative risk [RR] = 1.24, 95% confidence interval [CI] [1.05-1.47], P = 0.010) and reducing the incidence of Grade II or above nausea and vomiting (RR = 0.49, 95% CI [0.30-0.80], P = 0.005). Meanwhile, with cisplatin ranging from 80 to 120 mg/m 2 , no significant differences in efficiency (RR = 1.11, 95% CI [0.87-1.42], P = 0.390) and Grade II or above nausea and vomiting (RR = 0.88, 95% CI [0.71-1.10], P = 0.260) were obtained. In addition, Aidi injection combined with NP chemotherapy was superior to NP chemotherapy alone in improving the quality of life, alleviating Grade II or above leukopenia and thrombocytopenia.

CONCLUSIONSAidi injection combined with NP chemotherapy can enhance efficiency, improve the quality of life, and decrease adverse effects in patients with advanced NSCLC.

Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Carcinoma, Non-Small-Cell Lung ; drug therapy ; psychology ; Cisplatin ; administration & dosage ; Drugs, Chinese Herbal ; administration & dosage ; Humans ; Injections ; Lung Neoplasms ; drug therapy ; psychology ; Publication Bias ; Quality of Life ; Vinblastine ; administration & dosage ; analogs & derivatives