Purpose: Chronic myeloid leukemia (CML) in children, adolescents, and young adults is rare and differs from older adults. This study evaluated the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) in young Korean CML patients during the tyrosine kinase inhibitor (TKI) era. Materials and Methods: A retrospective analysis of 35 CML patients aged < 40 years who underwent allogeneic HSCT from 2009 to 2019 was conducted using Korean Blood and Marrow Transplantation Registry data. Patients were grouped by age < 20 years at HSCT (group 1, n=15) and 20-40 years at HSCT (group 2, n=20). Survival outcomes including overall survival (OS), relapse-free survival (RFS), and event-free survival (EFS) were analyzed using the Kaplan-Meier method. Results: The median time between diagnosis and HSCT was 8.9 months. All the patients achieved engraftment but platelet recovery was significantly slower in group 1 (p=0.034). Acute and chronic graft-versus-host disease occurred in 54.3% and 34.3%, respectively. Five-year OS, RFS, and EFS rates of total patients were 66.8%, 50.8%, and 47.6%, with better OS was observed in group 1 by multivariable analysis (p=0.048). Disease status at HSCT was a significant predictor of OS (p=0.028), RFS (p=0.003), and EFS (p=0.004). Disease progression occurred in 13 out of 35 patients (37.1%); treatment-related mortality accounted for 63.6% of deaths (7 out of 11). Conclusion When performed at a younger age, allogeneic HSCT result in superior outcome in CML. Achieving remission before HSCT is critical for improved outcomes, highlighting the importance of pretransplant remission via optimal TKI strategies and minimal residual disease monitoring.

BACKGROUND: The role of the phosphatidylinositol-3 kinase signaling pathway in the development of acral melanoma has recently gained evidence. Phosphatase and tensin homologue (PTEN), one of the key molecules in the pathway, acts as a tumor suppressor through either an Akt-dependent or Akt-independent pathway. Akt accelerates degradation of p53. OBJECTIVE: We assessed the expression of PTEN, phospho-Akt (p-Akt), and p53 by immunohistochemistry in benign acral nevi, acral dysplastic nevi, and acral melanomas in the radial growth phase and with a vertical growth component. METHODS: Ten specimens in each group were included. Paraffin-embedded specimens were immunostained with antibodies for PTEN, p-Akt, and p53. We scored both the staining intensity and the proportion of positive cells. The final score was calculated by multiplying the intensity score by the proportion score. RESULTS: All specimens of benign acral nevi except one showed some degree of PTEN-negative cells. The numbers of p-Akt and p53-positive cells were higher in acral dysplastic nevi and melanoma than in benign nevi. P-Akt scores were 1.7, 1.8, 2.6, and 4.4, and p53 scores were 2.0, 2.1, 3.8, and 4.1 in each group. PTEN and p-Akt scores in advanced acral melanoma were higher than in the other neoplasms. CONCLUSION: The expression of PTEN was decreased and the expression of p-Akt was increased in acral melanoma, especially in advanced cases. The PTEN-induced pathway appears to affect the late stage of melanomagenesis. Altered expression of p-Akt is thought to be due to secondary changes following the loss of PTEN.
Antibodies ; Dysplastic Nevus Syndrome* ; Immunohistochemistry ; Melanoma ; Nevus* ; Phosphotransferases

BACKGROUND: A variety of dermatological disorders develop in hospitalized patients and the need for dermatological consultations is on the rise. OBJECTIVE: We analyzed 2 years of dermatological consultation data from a tertiary medical center in Korea and compared dermatological problems among individual departments. METHODS: We reviewed 3,102 dermatological inpatient consultations by performing a retrospective chart review. RESULTS: Contact dermatitis (11.4%), drug eruption (9.6%), tinea pedis (5.5%), xerotic eczema (5.3%), and seborrheic dermatitis (5.2%) were the 5 dermatological disorders that were most commonly referred to dermatologists. Patients in the allergy and pulmonology departments had higher rates of drug eruptions. Endocrinology-admitted patients, especially diabetic patients, frequently complained of foot dermatitis. The cardiology, gastroenterology, and hemato-oncology departments referred many patients with purpuric dermatosis to dermatologists. Patients from the surgical departments consulted more frequently consulted contact dermatitis and drug eruptions. The neurology and rehabilitation medicine departments referred more seborrheic dermatitis patients than other departments. Pediatric patients commonly consulted for atopic dermatitis, viral exanthemata, and seborrheic dermatitis. Regardless of department, patients who suffered from severe illness and underwent major surgery developed herpes zoster and cutaneous fungal infections more frequently than other patients. Personal hygiene was closely related to the development of follicular disorders. CONCLUSION: Dermatologists should be familiar with the diverse dermatological complaints of patients admitted to various departments and be actively involved in the diagnosis, treatment, and education of doctors and patients to improve the quality of inpatient care.
Cardiology ; Dermatitis ; Dermatitis, Atopic ; Dermatitis, Contact ; Dermatitis, Seborrheic ; Diagnosis ; Drug Eruptions ; Eczema ; Education ; Foot ; Gastroenterology ; Herpes Zoster ; Humans ; Hygiene ; Hypersensitivity ; Inpatients* ; Korea ; Neurology ; Pulmonary Medicine ; Referral and Consultation ; Rehabilitation ; Retrospective Studies ; Skin Diseases ; Tinea Pedis

Hemophagocytic lymphohistiocytosis (HLH) is a fatal, systemic inflammatory syndrome that characteristically presents hemophagocytic histiocytes infiltrating into various organs. Common features of HLH are abrupt onset of high fever, malaise, lymphadenopathy, hepatosplenomegaly, and abnormal laboratory findings including pancytopenia, hypertriglyceridemia, hypofibrinogenemia, and high ferritin levels. Cutaneous manifestations, which occur in 6~65% of patients, can develop at any moment in the clinical course of the syndrome. Most skin eruptions in HLH patients are generalized, transient maculopapular rashes. We describe an HLH patient who first manifested cutaneous symptoms as a large purpuric patch on her thigh. A skin biopsy found high levels of hemophagocytic histiocytes.
Biopsy ; Exanthema ; Ferritins ; Fever ; Histiocytes ; Humans ; Hypertriglyceridemia ; Lymphatic Diseases ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia ; Purpura ; Skin ; Thigh

BACKGROUND: Diagnostic value of Bone marrow (BM) biopsies for fever of unknown origin (FUO) remain controversial and BM biopsies are difficult to perform in young patients. Our study aimed to elucidate the diagnostic yield of BM biopsies in FUO patients of all age, particularly for diagnosing hematological malignant diseases.METHODS: The medical records of 150 patients, hospitalized between January 1, 2008 and June 30, 2013, who underwent BM biopsies were evaluated to determine the cause of FUO. FUO was defined as fever (38.3degrees C, 101) either on several occasions during the 3 hospital days without a clear cause, after 1 week of invasive investigation, or after 3 outpatient visits. BM-specific diagnoses included those determined by BM biopsies (i.e., leukemia, lymphoma, myeloproliferative disease, myelodysplastic syndrome, aplastic anemia, and hemophagocytic lymphohistiocytosis).RESULTS: The final diagnoses of 24 patients (16%) were determined by BM biopsies; the majority included hematologic diseases and malignant neoplasms. Low hemoglobin levels, thrombocytopenia, bicytopenia, increased Lactate dehydrogenase (LDH) and ferritin levels, and ultrasonographic/computed tomographic abnormalities were significant risk factors (P<0.05). The young patient group (<18 years old) was safer from the tendency of BM biopsy diagnosis compared to adult patient group (>40 years old).CONCLUSION: Some laboratory abnormalities were related to the BM biopsy diagnostic yield. Furthermore, pediatric age was an important factor for deciding to do not perform excessive BM biopsies in FUO cases.
Adult ; Anemia, Aplastic ; Biopsy ; Bone Marrow ; Child ; Diagnosis ; Ferritins ; Fever of Unknown Origin ; Fever ; Hematologic Diseases ; Hematologic Neoplasms ; Humans ; L-Lactate Dehydrogenase ; Leukemia ; Lymphoma ; Medical Records ; Myelodysplastic Syndromes ; Outpatients ; Risk Factors ; Thrombocytopenia

Necrobiosis lipoidica is a chronic granulomatous disease that has a well-known association with diabetes mellitus. It usually affects the bilateral lower legs of middle-aged women. Over time, the clinical features vary from red-brown papules to sharply demarcated yellow-brown plaque with atrophic center. Some of the lesions may become ulcerated, especially in diabetic patients. A 61-year-old Korean female with a history of diabetes, ischemic heart disease, and pancreatic cancer was referred to our clinic for evaluation of the painful papulovesicles on her left lower leg. Under the assumptive diagnosis of herpes zoster, we immediately started an antiviral agent. However, her skin lesions rapidly increased in size and in number, and progressed to the multiple ulcerations in 9 days. Histopathologic examination showed several poorly circumscribed necrobiotic granulomas in the deep dermis and subcutis levels of the skin. In addition, prominent vascular ectasia and vasculitis were observed. We diagnosed the patient as having necrobiotic lipoidica, and treated her with oral dapsone, colchicine, phentoxifylline and antiplatelets. Her leg ulcerations healed after four weeks of treatment.
Colchicine ; Dapsone ; Dermis ; Diabetes Mellitus ; Dilatation, Pathologic ; Female ; Granuloma ; Granulomatous Disease, Chronic ; Herpes Zoster ; Humans ; Leg ; Leg Ulcer ; Myocardial Ischemia ; Necrobiosis Lipoidica ; Necrobiotic Disorders ; Pancreatic Neoplasms ; Skin ; Ulcer ; Vasculitis

Streptococcus bovis, a group D non-enterococcal organism has recently received increased attention, especially for its role as a cause of infective endocarditis and associated colorectal neoplasm. Infectious endocarditis due to group D streptococci include two non-enterococcal species, S. bovis and S. equinas, which may be mistaken for enterococci in clinical laboratory. However, S. bovis is readily distinguished from the enterococci by screen with bile-esculin hydrolysis and growth in 6.5% NaCl broth. Although endocarditis caused by S. bovis or enterococci share common clinical findings, therapeutically and prognostically, S. bovis endocarditis more resembles infection with viridans group organism. Also the infection of S. bovis significantly increased the prevalence of colorectal cancer in previous report. As discussed above, the patients with S. bovis endocarditis are carried out study of colorectal cancer. We report a case of endocarditis with colon cancer caused by S. bovis in 54 year old female.
Colon* ; Colonic Neoplasms* ; Colorectal Neoplasms ; Endocarditis* ; Female ; Humans ; Hydrolysis ; Middle Aged ; Prevalence ; Streptococcus bovis* ; Streptococcus*

Hemangioma is benign tumor that represent an fail in development of the vascular system, network or retiform stage. As with hemangioma elsewhere, spontaneous regression may occur. Histologically capillary hemangioma has a diagnostic lobular arrangement of capillaries and almost all of them are located in nasal or oral mucous membranes. But, as we know, there is no report of hemangioma located in the trachea in Korea, so we report a case of tracheal hemangioma which was manifested massive hemoptysis.
Capillaries ; Hemangioma* ; Hemangioma, Capillary ; Hemoptysis* ; Korea ; Laser Therapy ; Mucous Membrane ; Trachea