Objective: To explore the stem cell collection rate and efficacy and safety of patients aged 70 and below with newly diagnosed multiple myeloma (MM) treated with the VRD (bortezomib, lenalidomide and dexamethasone) regimen followed by autologous stem cell transplantation (ASCT). Methods: Retrospective case series study. The clinical data of 123 patients with newly diagnosed MM from August 1, 2018, to June 30, 2020, at the First Affiliated Hospital of Soochow University and Suzhou Hopes Hematology Hospital, who were eligible for VRD regimen sequential ASCT, were collected. The clinical characteristics, efficacy after induction therapy, mobilization regimen of autologous stem cells, autologous stem cell collection rate, and side effects and efficacy of ASCT were retrospectively analyzed. Results: Of the 123 patients, 67 were males. The median patient age was 56 (range: 31-70) years. Patients with IgG, IgA, IgD, and light-chain types accounted for 47.2% (58/123), 23.6% (29/123), 3.2% (4/123), and 26.0% (32/123) of patients, respectively. In addition, 25.2% (31/123) of patients had renal insufficiency (creatinine clearance rate<40 ml/min). Patients with Revised-International Staging System (R-ISS) Ⅲ accounted for 18.2% (22/121) of patients. After induction therapy, the rates of partial response and above, very-good partial response (VGPR) and above, and complete response (CR)+stringent CR were 82.1% (101/123), 75.6% (93/123), and 45.5% (56/123), respectively. Overall, 90.3% (84/93) of patients were mobilized with cyclophosphamide+granulocyte colony-stimulating factor (G-CSF) and 8 patients with G-CSF or G-CSF+plerixafor due to creatinine clearance rate<30 ml/min and one of them was mobilized with DECP (cisplatin, etoposide, cyclophosphamide and dexamethasone)+G-CSF for progressive disease. The rate of autologous stem cell collection (CD34⁺cells≥2×10⁶/kg) after four courses of VRD regimen was 89.1% (82/92), and the rate of collection (CD34⁺cells≥5×10⁶/kg) was 56.5% (52/92). Seventy-seven patients treated with the VRD regimen sequential ASCT. All patients had grade 4 neutropenia and thrombocytopenia. Among the nonhematologic adverse events during ASCT, the highest incidence was observed for gastrointestinal reactions (76.6%, 59/77), followed by oral mucositis (46.8%, 36/77), elevated aminotransferases (44.2%, 34/77), fever (37.7%, 29/77), infection (16.9%, 13/77) and heart-related adverse events (11.7%, 9/77). Among the adverse events, grade 3 adverse events included nausea (6.5%, 5/77), oral mucositis (5.2%, 4/77), vomiting (3.9%, 3/77), infection (2.6%, 2/77), elevated blood pressure after infusion (2.6%, 2/77), elevated alanine transaminase (1.3%, 1/77), and perianal mucositis (1.3%, 1/77); there were no grade 4 or above nonhematologic adverse events. The proportion of patients who achieved VGPR and above after VRD sequential ASCT was 100% (75/75), and the proportion of patients who were minimal residual disease-negative (<10^-4 level) was 82.7% (62/75). Conclusion: In patients aged 70 and below with newly diagnosed MM treated with VRD induction therapy, the collection rate of autologous stem cells was good, and good efficacy and tolerability were noted after follow-up ASCT.
Male ; Humans ; Female ; Multiple Myeloma/diagnosis* ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Retrospective Studies ; Creatinine ; Hematopoietic Stem Cell Mobilization ; Transplantation, Autologous ; Dexamethasone/therapeutic use* ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Heterocyclic Compounds/therapeutic use* ; Bortezomib/therapeutic use* ; Cyclophosphamide/therapeutic use* ; Stomatitis/etiology*

OBJECTIVE: To study the clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, a polygenic and multifactorial autoinflammatory disease with unknown pathogenesis. METHODS: A retrospective analysis was performed on the medical data of 13 children with PFAPA syndrome. RESULTS: All 13 children had disease onset within the age of 3 years, with a mean age of onset of (14±10) months. They all had periodic fever, with 8-18 attacks each year. The mean interictal period of fever was (30±5) days. Pharyngitis, cervical adenitis, and aphthous stomatitis were the three cardinal symptoms, with incidence rates of 100% (13/13), 85% (11/13), and 38% (5/13) respectively. There were increases in white blood cells, C-reactive protein, and erythrocyte sedimentation rate during fever. Of all the 13 children, 6 underwent whole exome sequencing and 7 underwent panel gene detection for autoinflammatory disease, and the results showed single heterozygous mutations in the CONCLUSIONS For children with unexplained periodic fever with early onset accompanied by pharyngitis, cervical adenitis, aphthous stomatitis, elevated inflammatory indices, and good response to glucocorticoids, PFAPA syndrome should be considered. This disorder has good prognosis, and early diagnosis can avoid the long-term repeated use of antibiotics.
Child ; Child, Preschool ; Fever/etiology* ; Humans ; Infant ; Lymphadenitis/diagnosis* ; Pharyngitis/drug therapy* ; Pyrin ; Retrospective Studies ; Stomatitis, Aphthous/genetics*

PURPOSE: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center.METHODS: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed.RESULTS: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months–8.3 years). The median age of periodic fever onset was 3 years (range, 1–6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4–13.2 mg/dL) and 29 mm/hr (range, 16–49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy.CONCLUSIONS: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.
Adenoidectomy ; Anti-Bacterial Agents ; Blood Sedimentation ; C-Reactive Protein ; Child ; Diagnosis ; Fever ; Follow-Up Studies ; Hematologic Tests ; Humans ; Lymphadenitis ; Male ; Neutropenia ; Pharyngitis ; Pharynx ; Prednisolone ; Retrospective Studies ; Seoul ; Stomatitis, Aphthous ; Tonsillectomy ; Yemen

PURPOSE: Although Bednar's aphthae are common and regress spontaneously, these lesions may lead to feeding intolerance and are often misdiagnosed, rendering examinations useless. This study sheds new light on the clinical features of Bednar's aphthae. METHODS: Sixteen neonates and infants were newly diagnosed with Bednar's aphthae via routine health check-ups in an outpatient clinic. Medical records were retrospectively reviewed, and the following parameters were analyzed; sex, gestational age, birth weight, mode of delivery, and perinatal problems. A physical examination was carried out during the next outpatient visit to examine the healing process and check for the existence of scars or complications. RESULTS: Initial presentation included changes in feeding habits (n=10), longer feeding time, reduced intake, and increased irritability. In 6 patients, Bednar's aphthae were discovered incidentally, without prior symptoms. Feeding posture and method of feeding are important causes of Bednar's aphthae. Eleven patients were fed in a horizontal position, whereas 5 patients were fed in a semiseated position. Fifteen patients were bottle-fed, whereas 1 patient was exclusively breastfed. After correcting the feeding position, the ulcerative lesions disappeared within 1 month of diagnosis. During the follow-up period, lesions did not recur in any of the patients. CONCLUSION: This study suggests that Bednar's aphthae are caused by mechanical pressure. A diagnosis of Bednar's aphthae should be considered when lesions are found on the palate of infants and when symptoms seem to be feeding related. Proper education of parents can both treat Bednar's aphthae and easily prevent its recurrence.
Ambulatory Care Facilities ; Birth Weight ; Cicatrix ; Diagnosis ; Education ; Follow-Up Studies ; Gestational Age ; Humans ; Infant* ; Infant, Newborn ; Medical Records ; Mouth Mucosa ; Outpatients ; Palate ; Parents ; Physical Examination ; Posture ; Recurrence ; Retrospective Studies ; Stomatitis, Aphthous* ; Ulcer

In this case study, the process of nutritional diagnosis and intervention conducted at a hospital on a malnourished patient who underwent treatment for a chronic illness (chemotherapy for cancer treatment) was recorded. The patient received his first round of chemotherapy for colorectal cancer, and then a second round after the cancer metastasized to the liver. The patient was malnourished and had experienced weight loss (17% loss in the most recent 3 months) due to side effects of chemotherapy including stomatitis, nausea, and vomiting. Nutritional diagnosis and intervention via the nutrition care process were implemented through two screening rounds, and the quantity of oral intake increased from 28% to 62% of the recommended daily intake. The patient required continuous monitoring and outpatient care after hospital discharge. It is speculated that if a more active patient education and dietary regimen with respect to chemotherapy side effects had been offered after the patient's first chemotherapy cycle, it might have been possible to treat ingestion problems due to stomatitis during the second cycle of chemotherapy and prevent the weight loss. Henceforth, patients receiving chemotherapy should be educated about nutrition management methods and monitored continuously to prevent malnutrition.
Ambulatory Care ; Chronic Disease ; Colorectal Neoplasms ; Diagnosis ; Drug Therapy* ; Eating ; Humans ; Liver ; Malnutrition ; Mass Screening ; Nausea ; Patient Education as Topic ; Recommended Dietary Allowances ; Stomatitis ; Vomiting ; Weight Loss

Behcet's disease is an inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. A few cases of hematologic disease in patients with Behcet's disease have been reported in the literature. However, acute precursor T cell lymphoblastic leukemia has never been described in association with Behcet's disease. We recently encountered a case of acute precursor T cell lymphoblastic leukemia in a 62-year-old man with a prior diagnosis of Behcet's disease. The patient presented with febrile neutropenia and his bone marrow biopsy revealed acute precursor T cell lymphoblastic leukemia. He was scheduled to undergo therapeutic chemotherapy, but unfortunately he died from pneumonia prior to treatment.
Biopsy ; Bone Marrow ; Diagnosis ; Drug Therapy ; Febrile Neutropenia ; Hematologic Diseases ; Humans ; Middle Aged ; Pneumonia ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* ; Skin ; Stomatitis, Aphthous ; Ulcer ; Uveitis

Severe congenital neutropenia (SCN) is a heterogeneous group of disorders with a defect in granulopoiesis causing marked neutropenia and severe bacterial infections. A 17-month-old girl (patient 1) was admitted due to cervical lymphadenitis caused by methicillin-resistant Staphylococcus aureus, with neutropenia. She had Pseudomonas aeruginosa sepsis and peritonitis with perforated appendicitis at 8-month of age. Her sister, a 37-month-old girl (patient 2), had recurrent stomatitis with profound neutropenia, and her mother, a 32-yr-old woman (patient 3), had had recurrent stomatitis until her early 20s with neutropenia. We found an ELANE gene mutation (c.597+1G > A) from them in direct DNA sequencing analysis. Patients 1 and 2 did not respond to granulocyte colony stimulating factor and patient 1 was treated with prolonged antibiotics and excision. We demonstrated inherited SCN cases showing different severity even with the same mutation of the ELANE gene in a family.
Adult ; Child, Preschool ; DNA Mutational Analysis ; Female ; Granulocyte Colony-Stimulating Factor/therapeutic use ; Humans ; Infant ; Leukocyte Elastase/*genetics ; Methicillin-Resistant Staphylococcus aureus/isolation & purification ; Mutation/genetics ; Neutropenia/*congenital/diagnosis/drug therapy/genetics ; Pedigree ; *Phenotype ; Polymorphism, Single Nucleotide ; Recurrence ; Staphylococcal Infections/diagnosis/microbiology ; Stomatitis/diagnosis ; Tomography, X-Ray Computed

PURPOSE: This study was designed to investigate the difference of oral mucositis in hospitalized cancer patients receiving chemotherapy according to characteristics. METHODS: Data were collected from March 1 to April 25 2012 using questionnaires, and the participants were 177. The Oral Assessment Guide (OAG) by observation and The Oral Mucositis Daily Questionnaire (OMDQ) by self-report were used to measure oral mucositis. The data were analyzed using SPSS 18.0. RESULTS: Moderate to severe oral mucositis measured by OAG observation were 94.9% in hospitalized cancer patients receiving chemotherapy, frequently complaints of mouth and throat soreness measured by OMDQ was 42.4%, and intensity of mouth and throat soreness measured by OMDQ was 0.65 out of 10. There were statistically significant differences in the prevalence of oral mucositis according to nutrition state, frequency of chemotherapy, experience of oral examination, combination treatment, and number of chemotherapeutic agents. CONCLUSION: Many hospitalized cancer patients receiving chemotherapy experienced oral mucositis. Cancer patients receiving chemotherapy with poor nutritional status, more frequent chemotherapy and combination treatments should have a more meticulous oral care. So there needs to be an appropriate assessment tool and prevention protocol for oral mucositis that considers related factors.
Diagnosis, Oral ; Humans ; Mouth ; Nutritional Status ; Pharynx ; Prevalence ; Stomatitis ; Surveys and Questionnaires

Consensus ; Diagnosis, Differential ; Glucocorticoids ; therapeutic use ; Humans ; Practice Guidelines as Topic ; Recurrence ; Stomatitis, Aphthous ; diagnosis ; therapy

Drug hypersensitivity syndrome (DHS) is an idiosyncratic systemic reaction to a drug. The clinical presentation of this syndrome comprises a diverse spectrum, ranging from mild to fulminating organ failure. Nonspecific gastrointestinal symptoms are common in DHS, but severe morbidities and mortalities attributed to gut disease in DHS are rarely described. We present a case of DHS with significant gastrointestinal symptoms of prolonged profuse watery diarrhoea and persistent hypokalaemia requiring judicious intravenous water and electrolyte replacement. The symptoms resolved only after the introduction of intravenous hydrocortisone. It is important to consider intravenous corticosteroids if the gastrointestinal system is involved, as accelerated gut motility and mucosal damage would affect absorption of oral medications. Supportive treatment with the monitoring of fluid and electrolytes status and judicious replacement remains fundamental in the management of DHS patients with gut involvement.
Amoxicillin-Potassium Clavulanate Combination ; therapeutic use ; Diarrhea ; complications ; diagnosis ; Drug Eruptions ; diagnosis ; drug therapy ; Drug Hypersensitivity Syndrome ; complications ; diagnosis ; Edema ; chemically induced ; Electrolytes ; Female ; Gastrointestinal Diseases ; chemically induced ; complications ; Humans ; Hydrocortisone ; therapeutic use ; Middle Aged ; Otitis Media ; complications ; drug therapy ; Prednisolone ; therapeutic use ; Stomatitis ; chemically induced