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1.
Article in English | WPRIM | ID: wpr-831569

ABSTRACT

Background@#Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. @*Methods@#We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. @*Results@#A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. @*Conclusion@#This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

2.
Article | WPRIM | ID: wpr-831549

ABSTRACT

Background@#Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. @*Methods@#We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. @*Results@#A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. @*Conclusion@#In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

3.
Article | WPRIM | ID: wpr-831054

ABSTRACT

Purpose@#Loss of heterozygosity (LOH) at chromosomes 1p and 16q is a poor prognostic factor infavorable histology Wilms tumor (FHWT). This study investigated the prevalence of LOH at1p and 16q and evaluated its prognostic value in Korean children with FHWT. @*Materials and Methods@#We analyzed 101 FHWT patients who were diagnosed between 1996 and 2016 in KoreanSociety of Pediatric Hematology Oncology Group hospitals. Using paraffin-embedded kidneytissue samples sent from each center, we reviewed LOH at 1p and 16q in each patient andassessed the prognostic value of LOH status for clinical parameters affecting event-freesurvival (EFS). @*Results@#Of the 101 patients, 12 (11.9%) experienced recurrence; the 3-year EFS was 87.6%. LOHat 1p or 16q was detected in 19 patients (18.8%), with five having LOH at both 1q and 16q.The frequency of LOH at 1p was higher among younger patients (p=0.049), but there wasno difference in LOH prevalence according to tumor stage. In the multivariate analysis, LOHat 16q was a significant negative prognostic factor affecting EFS (3-year EFS, 73.7% vs.91.1%; hazard ratio, 3.95; p=0.037), whereas LOH at 1p was not (p=0.786). @*Conclusion@#LOH at 16q was a significant negative prognostic factor affecting outcome in Korean pediatricFHWT patients. Due to the small sample size of this study, large-scale multicenter trialsare warranted to investigate the prognostic value of LOH at 1p and 16q in Korean childrenwith FHWT.

4.
Article in English | WPRIM | ID: wpr-763514

ABSTRACT

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.


Subject(s)
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
5.
Article in English | WPRIM | ID: wpr-785307

ABSTRACT

The dose of CD34+ cells is known to influence the outcome of allogeneic peripheral blood stem cell (PBSC) and/or T-cell-depleted transplantation. A previous study proposed that 2×10⁶ CD34+ cells/kg is the ideal minimum dose for allogeneic transplantation, although lower doses did not preclude successful therapy. In the case we present here, CD34+ cells were collected from a matched sibling donor on the day of allogeneic hematopoietic stem cell transplantation; however, the number of cells was not sufficient for transplantation. Consequently, PBSCs were collected three additional times and were infused along with cord blood cells from the donor that were cryopreserved at birth. The cumulative dose of total nuclear cells and CD34+ cells was 15.9×10⁸ cells/kg and 0.95×10⁶ cells/kg, respectively. White blood cells from this patient were engrafted on day 12. In summary, we report successful engraftment after infusion of multiple low doses of CD34+ cells in a patient with severe aplastic anemia.


Subject(s)
Anemia, Aplastic , Cord Blood Stem Cell Transplantation , Fetal Blood , Hematopoietic Stem Cell Transplantation , Humans , Leukocytes , Parturition , Peripheral Blood Stem Cell Transplantation , Siblings , Stem Cells , Tissue Donors , Transplantation, Homologous
6.
Article in Korean | WPRIM | ID: wpr-717643

ABSTRACT

BACKGROUND: Langerhans cell histiocytosis (LCH) frequently involves the head and neck and increases the risk of central nervous system (CNS) involvement of LCH, such as central diabetes insipidus (CDI), when the craniofacial bones are involved. We analyzed risk factors and clinical features of CDI among patients with LCH involving the head and neck. METHODS: From January 1, 2000 to May 1, 2018, 63 patients with histologically confirmed LCH in the Department of Pediatrics, Ajou University Hospital were retrospectively analyzed. RESULTS: Forty eight cases (76.2%) of patients had head and neck involvement, and 9 cases (14.3%) in craniofacial bones at the time of initial diagnosis of LCH. CDI was found in 6 cases (9.5%) among all LCH patients, 6 cases (12.2%) among patients with head and neck involvement, and 3 cases (33.3%) among patients with craniofacial bone involvement. Three cases of CDI occurred at the time of initial LCH diagnosis, and another 3 cases occurred at the time of 2, 4, and 8 years after initial LCH diagnosis. Of the 6 CDI patients, 3 had CNS risk lesions and 3 had no CNS risk lesions, but all had multi-system involvement of LCH. CONCLUSION: CDI can occur even in patients with head and neck LCH without CNS risk lesions, if there are multisystem involvement of LCH. Patients with head and neck LCH may develop CDI over time, so continuous observations should be done while considering the occurrence of CDI.


Subject(s)
Central Nervous System , Diabetes Insipidus, Neurogenic , Diagnosis , Head , Histiocytosis, Langerhans-Cell , Humans , Neck , Pediatrics , Retrospective Studies , Risk Factors
7.
Article in English | WPRIM | ID: wpr-739807

ABSTRACT

While congenital muscular torticollis (CMT) can occur along with other conditions, such as clavicle fracture or brachial plexus injury, these conditions exist outside the sternocleidomastoid muscle (SCM). We present a rare case with concurrence of CMT and a malignant tumor inside the same SCM, along with serial clinical and radiological findings of the atypical features of CMT. The malignant tumor was in fact a low-grade fibromyxoid sarcoma. To the best of our knowledge, the current case is the first of a concurrent condition of CMT inside the SCM. This case suggests that concurrent conditions could exist either inside or outside the SCM with CMT. Therefore, a thorough evaluation of SCM is required when subjects with CMT display atypical features, such as the increase of mass or poor response to conservative therapy. In that case, appropriate imaging modalities, such as ultrasonogram or magnetic resonance imaging, are useful for differential diagnosis.


Subject(s)
Brachial Plexus , Clavicle , Diagnosis, Differential , Fibrosarcoma , Magnetic Resonance Imaging , Sarcoma , Torticollis , Ultrasonography
8.
Article in English | WPRIM | ID: wpr-714971

ABSTRACT

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disease characterized by fever and lymphadenopathy. The etiology of KFD is unknown, but an autoimmune cause has been suggested. Hashimoto thyroiditis is the most common autoimmune thyroid disorder in children and is known to be associated with other autoimmune diseases. Only a few cases of Hashimoto thyroiditis associated with KFD have been documented. We report a case of a 16-year-old girl who was first diagnosed with KFD and developed Hashimoto thyroiditis 2 years and 6 months later during her follow-up period. Physicians of patients with KFD should consider the possibility of autoimmune diseases like Hashimoto’s thyroiditis.


Subject(s)
Adolescent , Autoimmune Diseases , Child , Female , Fever , Follow-Up Studies , Hashimoto Disease , Histiocytic Necrotizing Lymphadenitis , Humans , Lymphatic Diseases , Thyroid Gland , Thyroiditis
9.
Korean Journal of Medicine ; : 541-545, 2017.
Article in Korean | WPRIM | ID: wpr-103596

ABSTRACT

Heavy proteinuria in the nephrotic range is an uncommon, often unrecognized manifestation of graft-versus-host disease (GVHD) following hematopoietic stem cell transplantation. A few isolated case reports have been published in the Korean literature involving a small number of patients who developed membranous nephropathy as GVHD after peripheral blood stem cell transplantation (PBSCT). A 17-year-old female was diagnosed with non-Hodgkin's lymphoma. Following remission, she underwent allogeneic PBSCT. Shortly thereafter, she developed acute GVHD, which was managed by medical therapy with prednisolone and cyclosporine. Approximately 13 months following PBSCT, the patient developed proteinuria without peripheral edema. Pulsed steroid therapy was initiated three times, but her condition did not improve. Twenty months after PBSCT, she developed nephrotic range proteinuria. A renal biopsy was performed, and the diagnosis was histologically consistent with membranous nephropathy. Because the response to steroids was not satisfactory, the dose of cyclosporine was increased. Approximately 3 months after renal biopsy, the proteinuria disappeared. Given the recent increase in the incidence of GVHD-mediated renal disease, in particular, renal biopsy is indispensable to the diagnosis of nephropathy and to the prevention of disease progression.


Subject(s)
Adolescent , Biopsy , Cyclosporine , Diagnosis , Disease Progression , Edema , Female , Glomerulonephritis, Membranous , Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Humans , Incidence , Lymphoma, Non-Hodgkin , Peripheral Blood Stem Cell Transplantation , Prednisolone , Proteinuria , Stem Cells , Steroids
10.
Article in English | WPRIM | ID: wpr-49314

ABSTRACT

This multicenter, prospective trial was conducted to develop an effective and safe reinduction regimen for marrow-relapsed pediatric acute lymphoblastic leukemia (ALL) by modifying the dose of idarubicin. Between 2006 and 2009, the trial accrued 44 patients, 1 to 21 years old with first marrow-relapsed ALL. The reinduction regimen comprised prednisolone, vincristine, L-asparaginase, and idarubicin (10 mg/m²/week). The idarubicin dose was adjusted according to the degree of myelosuppression. The second complete remission (CR2) rate was 72.7%, obtained by 54.2% of patients with early relapse < 24 months after initial diagnosis and 95.0% of those with late relapse (P = 0.002). Five patients entered remission with extended treatment, resulting in a final CR2 rate of 84.1%. The CR2 rate was not significantly different according to the idarubicin dose. The induction death rate was 2.3% (1/44). The 5-year event-free and overall survival rates were 22.2% ± 6.4% and 27.3% ± 6.7% for all patients, 4.2% ± 4.1% and 8.3% ± 5.6% for early relapsers, and 43.8% ± 11.4% and 50.0% ± 11.2% for late relapsers, respectively. Early relapse and slow response to reinduction chemotherapy were predictors of poor outcomes. In conclusion, a modified dose of idarubicin was effectively incorporated into the reinduction regimen for late marrow-relapsed ALL with a low toxic death rate. However, the CR2 rate for early relapsers was suboptimal, and the second remission was not durable in most patients.

11.
Article in Korean | WPRIM | ID: wpr-788616

ABSTRACT

BACKGROUND: Severe neutropenia is defined as an absolute neutrophil count (ANC) less than 0.5×109/L, which is known to increase the risk of serious bacterial infections. The aim of this study was to investigate characteristics, etiology and differences between transient and chronic severe neutropenia in children.METHODS: 204 children, who were diagnosed with severe neutropenia at the Ajou University Hospital during a 5-year period, were included in the study. Clinical and laboratory features were analyzed. The patients were classified as having transient severe neutropenia (TSN) if recovery occurred within 6 months of diagnosis, and chronic severe neutropenia (CSN) if the neutropenia persisted for 6 months or more.RESULTS: 184 (90.2%) patients with TSN and 20 (9.8%) patients with CSN were identified. Most of the TSN occurred in patients less than 2 year of age (75.5%) and rarely occurred in patients 5 years or older (5.4%). The most common cause of TSN was infection-related neutropenia (82.6%), and most of the associated infections were respiratory infections (44.6%). Compared to TSN, CSN patients were younger at diagnosis (1.00 vs. 0.71, P < 0.001), had a lower ANC at diagnosis (364.8 vs. 214.9, P < 0.001), lower ANC at nadir (356.0 vs. 50.0, P < 0.001), and higher platelet count (188×10⁹ vs. 308×10⁹, P < 0.001), monocyte count (491.5×10⁶ vs. 832.9×10⁶, P=0.010) and CRP (0.22 vs. 0.85, P=0.036).CONCLUSION: Most of the severe neutropenia occurred in children younger than 2 years of age, and virus infection was the most common cause of TSN.


Subject(s)
Bacterial Infections , Child , Diagnosis , Humans , Monocytes , Neutropenia , Neutrophils , Platelet Count , Respiratory Tract Infections
12.
Article in Korean | WPRIM | ID: wpr-788596

ABSTRACT

BACKGROUND: Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing leukemia. However, in some patients, leukemia does not become apparent despite significant number of blast cells in the peripheral blood. This condition is called Transient myeloproliferative disorder (TMD), and is a disease entity unique to DS newborns and defined as the morphologic detection of blasts in DS less than three months of age. The present study investigated whether there was a difference between leukemia and TMD, and determined prognostic and risk factors.METHODS: We collected blood samples from 317 patients of 433 DS confirmed patients. We found 18 patients who had blast cells in their peripheral blood.RESULTS: Twelve patients were positive for blasts during the neonate period, and only one patient progressed to leukemia. The other 11 patients were later diagnosed with TMD. Six more patients were later diagnosed with leukemia, therefore, 7 patients were diagnosed with leukemia in total. All patients diagnosed with leukemia had anemia at the time of diagnosis, which was not found in TMD patients. All leukemia patients developed their disease after three months of life. Acute Myeloid Leukemia (AML) patients had additional chromosome mutation to trisomy 21 when they were diagnosed.CONCLUSION: In patients with Down Syndrome, anemia at diagnosis and age of onset could be helpful in distinguishing TMD from acute leukemia. Cancerous mutations in the chromosomes of peripheral and marrow blast cells of Down syndrome patients may foreshadow acute leukemia.


Subject(s)
Age of Onset , Anemia , Bone Marrow , Child , Diagnosis , Down Syndrome , Humans , Infant, Newborn , Leukemia , Leukemia, Myeloid, Acute , Myeloproliferative Disorders , Risk Factors
13.
Article in Korean | WPRIM | ID: wpr-123908

ABSTRACT

BACKGROUND: Severe neutropenia is defined as an absolute neutrophil count (ANC) less than 0.5×109/L, which is known to increase the risk of serious bacterial infections. The aim of this study was to investigate characteristics, etiology and differences between transient and chronic severe neutropenia in children. METHODS: 204 children, who were diagnosed with severe neutropenia at the Ajou University Hospital during a 5-year period, were included in the study. Clinical and laboratory features were analyzed. The patients were classified as having transient severe neutropenia (TSN) if recovery occurred within 6 months of diagnosis, and chronic severe neutropenia (CSN) if the neutropenia persisted for 6 months or more. RESULTS: 184 (90.2%) patients with TSN and 20 (9.8%) patients with CSN were identified. Most of the TSN occurred in patients less than 2 year of age (75.5%) and rarely occurred in patients 5 years or older (5.4%). The most common cause of TSN was infection-related neutropenia (82.6%), and most of the associated infections were respiratory infections (44.6%). Compared to TSN, CSN patients were younger at diagnosis (1.00 vs. 0.71, P < 0.001), had a lower ANC at diagnosis (364.8 vs. 214.9, P < 0.001), lower ANC at nadir (356.0 vs. 50.0, P < 0.001), and higher platelet count (188×10⁹ vs. 308×10⁹, P < 0.001), monocyte count (491.5×10⁶ vs. 832.9×10⁶, P=0.010) and CRP (0.22 vs. 0.85, P=0.036). CONCLUSION: Most of the severe neutropenia occurred in children younger than 2 years of age, and virus infection was the most common cause of TSN.


Subject(s)
Bacterial Infections , Child , Diagnosis , Humans , Monocytes , Neutropenia , Neutrophils , Platelet Count , Respiratory Tract Infections
14.
Article in Korean | WPRIM | ID: wpr-197956

ABSTRACT

BACKGROUND: Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing leukemia. However, in some patients, leukemia does not become apparent despite significant number of blast cells in the peripheral blood. This condition is called Transient myeloproliferative disorder (TMD), and is a disease entity unique to DS newborns and defined as the morphologic detection of blasts in DS less than three months of age. The present study investigated whether there was a difference between leukemia and TMD, and determined prognostic and risk factors. METHODS: We collected blood samples from 317 patients of 433 DS confirmed patients. We found 18 patients who had blast cells in their peripheral blood. RESULTS: Twelve patients were positive for blasts during the neonate period, and only one patient progressed to leukemia. The other 11 patients were later diagnosed with TMD. Six more patients were later diagnosed with leukemia, therefore, 7 patients were diagnosed with leukemia in total. All patients diagnosed with leukemia had anemia at the time of diagnosis, which was not found in TMD patients. All leukemia patients developed their disease after three months of life. Acute Myeloid Leukemia (AML) patients had additional chromosome mutation to trisomy 21 when they were diagnosed. CONCLUSION: In patients with Down Syndrome, anemia at diagnosis and age of onset could be helpful in distinguishing TMD from acute leukemia. Cancerous mutations in the chromosomes of peripheral and marrow blast cells of Down syndrome patients may foreshadow acute leukemia.


Subject(s)
Age of Onset , Anemia , Bone Marrow , Child , Diagnosis , Down Syndrome , Humans , Infant, Newborn , Leukemia , Leukemia, Myeloid, Acute , Myeloproliferative Disorders , Risk Factors
15.
Article in English | WPRIM | ID: wpr-218779

ABSTRACT

Idiopathic pulmonary hemosiderosis (IPH) is a rare respiratory disease with an unknown etiology, and is diagnosed with laboratory, radiology, and pathology tests. Chief complaints of IPH include hemoptysis, cough, and dyspnea. Since it is considered an immune-mediated disease, the first line of treatment is systemic corticosteroid therapy. The three cases reported here showed a decrease in ferritin level and improvement in the hemoglobin level with prednisolone treatment. However, long-term corticosteroid therapy may cause several side effects, particularly growth retardation and obesity, which can affect growing children. In the present study, all patients had cushingoid symptoms and obesity. Therefore, we switched to deflazacort (DFZ), which has lesser side-effects of weight gain. This report describes clinical courses of the disease and comparison of body mass index of three patients with IPH who took DFZ instead of prednisolone. DFZ was effective for IPH, and is useful for weight gain reduction.


Subject(s)
Body Mass Index , Child , Cough , Dyspnea , Ferritins , Hemoptysis , Hemosiderosis , Humans , Obesity , Pathology , Prednisolone , Weight Gain
16.
Article in English | WPRIM | ID: wpr-30881

ABSTRACT

Langerhans cell histiocytosis (LCH) is a rare disease having an unknown etiology, but characterized by a disorder of antigen presenting cells and the mononuclear phagocytic system. Neonatal LCH, defined as LCH presenting within the first 4 weeks of life, accounts for less than 5-6% of the total LCH cases. Skin involvement of neonatal LCH tends to regress spontaneously, as has been shown in Hashimoto-Pritzker syndrome. We report a case of LCH that presented as whole body, polymorphic, generalized, erythematous, yellowish plaques at birth, with no evidence of other organ involvement. These skin lesions regressed spontaneously within 14 days. Two weeks later, however, the patient presented with hepatosplenomegaly, fever, hyperbilirubinemia, anemia and leukocytosis. LCH with liver involvement was shown on abdominal MRI findings, and the patient received systemic chemotherapy. Although neonatal LCH may initially only show skin manifestations, it should be followed up closely and periodically for other organ involvement.


Subject(s)
Anemia , Antigen-Presenting Cells , Drug Therapy , Fever , Histiocytosis, Langerhans-Cell , Humans , Hyperbilirubinemia , Infant, Newborn , Leukocytosis , Liver , Magnetic Resonance Imaging , Parturition , Rare Diseases , Skin Manifestations , Skin
17.
Radiation Oncology Journal ; : 305-312, 2016.
Article in English | WPRIM | ID: wpr-33370

ABSTRACT

PURPOSE: The objective of this prospective study was to evaluate the relationship between the circulating lymphocyte subpopulation counts during preoperative chemoradiotherapy (CRT) and tumor response in locally advanced rectal cancer. MATERIALS AND METHODS: From August 2015 to June 2016, 10 patients treated with preoperative CRT followed by surgery were enrolled. Patients received conventional fractionated radiotherapy (50.4 Gy) with fluorouracil-based chemotherapy. Surgical resection was performed at 4 to 8 weeks after the completion of preoperative CRT. The absolute blood lymphocyte subpopulation was obtained prior to and after 4 weeks of CRT. We analyzed the association between a tumor response and change in the lymphocyte subpopulation during CRT. RESULTS: Among 10 patients, 2 (20%) had evidence of pathologic complete response. In 8 patients with clinically node positive, 4 (50%) had nodal tumor response. All lymphocyte subpopulation counts at 4 weeks after CRT were significantly lower than those observed during pretreatment (p < 0.01). A high decrease in natural killer (NK) cell, count during CRT (baseline cell count − cell count at 4 weeks) was associated with node down staging (p = 0.034). CONCLUSION: Our results suggest that the change of lymphocyte subset to preoperative CRT may be a predictive factor for tumor response in rectal cancer.


Subject(s)
Cell Count , Chemoradiotherapy , Drug Therapy , Humans , Killer Cells, Natural , Lymphocyte Subsets , Lymphocytes , Prospective Studies , Radiotherapy , Rectal Neoplasms
18.
Article in English | WPRIM | ID: wpr-170061

ABSTRACT

PURPOSE: The objective of this study was to explore the relationship between the circulating lymphocyte level during preoperative chemoradiotherapy (CRT) and pathologic complete response (pCR) in locally advanced rectal cancer. MATERIALS AND METHODS: From May 2010 to May 2013, 52 patients treated with preoperative CRT followed by surgery, were analysed. Patients received conventional fractionated radiotherapy (50-54 Gy) with fluorouracil-based chemotherapy. Surgical resection was performed at 4 to 8 weeks after the completion of preoperative CRT. Absolute blood lymphocyte counts and their relative percentage in total white blood cell counts were obtained from complete blood count tests performed prior to and after 4, 8, and 12 weeks of CRT. We analysed the association between achieving pCR and change in blood lymphocyte level during CRT, as well as clinical parameters. RESULTS: Among 52 patients, 14 (26.9%) had evidence of pCR. Sustaining the blood lymphocyte count during CRT (lymphocyte count at 4 weeks/baseline lymphocyte count > 0.35; odds ratio, 8.33; p=0.02) and initial carcinoembryonic antigen < 4.4 ng/mL (odds ratio, 6.71; p=0.03) were significantly associated with pCR in multivariate analyses. CONCLUSION: Sustaining blood lymphocyte count during preoperative CRT was predictive for pCR in rectal cancer. Further studies are warranted to investigate the association between pathologic responses and circulating lymphocyte count with its subpopulation during preoperative CRT.


Subject(s)
Blood Cell Count , Carcinoembryonic Antigen , Chemoradiotherapy , Drug Therapy , Humans , Leukocyte Count , Lymphocyte Count , Lymphocytes , Multivariate Analysis , Neoadjuvant Therapy , Odds Ratio , Polymerase Chain Reaction , Radiotherapy , Rectal Neoplasms
19.
Article in Korean | WPRIM | ID: wpr-97104

ABSTRACT

BACKGROUND: Hepatic hemagioendothelioma in neonatal period was rarely seen, so standard treatment does not established yet. METHODS: A retrospective analysis of patients with neonatal hepatic hemangioendothelioma at Ajou University Hospital between 2001 and 2016 was performed. RESULTS: Six patients with hepatic hemangioendothelioma in neonatal period were founded. Mean age at diagnosis was 6.1 days (range, 1-26 days). Three patients have no symptoms; diagnostic approach was prenatal ultrasonography in 2 patients, and incidental abnormal ultrasonographic findings in 1 patient, but the other 3 patients have hepatomegaly and/or congestive heart failure. Three patients were observed without treatment and the other 3 patients received medical and/or surgical treatment. Three patients of those who did not receive treatment became spontaneous regression. Of the other 3 patients, 1 patient achieved complete tumor disappearance after surgical resection, another 1 patient achieved to decrease tumor size with interferon-alpha treatment for 6 months and then had complete resolution of tumor after partial liver lobectomy, and other 1 patient who received hepatic artery embolization decreased in the size and number of lesions and then regressed gradually. CONCLUSION: Asymptomatic patients with neonatal hepatic hemangioendothelioma could have spontaneous remission, but patients with symptoms such as hepatomegaly with congestive heart failure or thrombocytopenia needed to be applied with medical and/or surgical treatment.


Subject(s)
Diagnosis , Heart Failure , Hemangioendothelioma , Hepatic Artery , Hepatomegaly , Humans , Interferon-alpha , Liver , Remission, Spontaneous , Retrospective Studies , Thrombocytopenia , Ultrasonography, Prenatal
20.
Article in English | WPRIM | ID: wpr-788577

ABSTRACT

Langerhans cell histiocytosis (LCH) is a rare disease having an unknown etiology, but characterized by a disorder of antigen presenting cells and the mononuclear phagocytic system. Neonatal LCH, defined as LCH presenting within the first 4 weeks of life, accounts for less than 5-6% of the total LCH cases. Skin involvement of neonatal LCH tends to regress spontaneously, as has been shown in Hashimoto-Pritzker syndrome. We report a case of LCH that presented as whole body, polymorphic, generalized, erythematous, yellowish plaques at birth, with no evidence of other organ involvement. These skin lesions regressed spontaneously within 14 days. Two weeks later, however, the patient presented with hepatosplenomegaly, fever, hyperbilirubinemia, anemia and leukocytosis. LCH with liver involvement was shown on abdominal MRI findings, and the patient received systemic chemotherapy. Although neonatal LCH may initially only show skin manifestations, it should be followed up closely and periodically for other organ involvement.


Subject(s)
Anemia , Antigen-Presenting Cells , Drug Therapy , Fever , Histiocytosis, Langerhans-Cell , Humans , Hyperbilirubinemia , Infant, Newborn , Leukocytosis , Liver , Magnetic Resonance Imaging , Parturition , Rare Diseases , Skin Manifestations , Skin
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