Benedikt syndrome is characterized by ipsilateral ophthalmoplegia with contralateral hemichorea due to a midbrain lesion. A 67-year-old male with Benedikt syndrome underwent corpectomy at L1 and anterolateral interbody fusion at T12-L2 due to pathologic bursting fracture at L1 involving multiple myeloma. He had a history of traumatic subarachnoid hemorrhage and subdural hemorrhage 8 months before surgery. Magnetic resonance image of the brain revealed intracranial hemorrhage from thalamus to midbrain. Target controlled infusion with propofol and remifentanil were administered for anesthetic induction and maintenance and close hemodynamic and neurologic monitoring led to successful anesthetic management.
Aged ; Anesthesia ; Brain ; Hematoma, Subdural ; Hemodynamics ; Humans ; Intracranial Hemorrhages ; Male ; Mesencephalon ; Multiple Myeloma* ; Ophthalmoplegia ; Propofol ; Spine* ; Subarachnoid Hemorrhage, Traumatic ; Thalamus

It has been suggested that dendritic cells (DCs) are critical antigen presenting cells for eosinophilic airway inflammation in a mouse model of asthma, and cysteinyl leukotrienes may play a role in DC trafficking in asthmatics. We investigated whether the number of DCs is increased in the induced sputum of both atopic and nonatopic asthmatics and is related to activated eosinophil count in the sputum. Sputum was induced by inhalation of hypertonic saline in 9 atopic and 12 nonatopic asthmatics and 10 nonatopic normal controls, and differential cell counts were performed. DCs and activated eosinophils were identified by immunocytochemistry with monoclonal antibodies (anti-CD1a and EG2, respectively). There were significantly higher percentages of eosinophils, EG2+ cells, and CD1a+ DC in the sputum of atopic and nonatopic asthmatics compared with normal controls, respectively. In asthmatics, the percentage of CD1a+ DC was significantly correlated with that of EG2+ cells (Rs=0.62, p=0.004). We demonstrated that the increased number of DCs was evident in the induced sputum of both atopic and nonatopic asthmatics, and the DC number was related to the activated eosinophil count, which suggests that DCs may contribute to the ongoing eosinophilic inflammation in asthmatic airways, and vice versa.
Adult ; Aged ; Antigens, CD1/analysis ; Asthma/*immunology/pathology ; Comparative Study ; Dendritic Cells/*immunology ; Eosinophil Granule Proteins/analysis ; Eosinophils/cytology/*immunology ; Female ; Humans ; Immunohistochemistry ; Leukocyte Count ; Male ; Middle Aged ; Research Support, Non-U.S. Gov't ; Sputum/cytology/*immunology

Interdigitating dendritic cell sarcoma (IDCS) is a very rare and aggressive neoplasm that arises from antigen presenting cells. IDCS usually involves lymph nodes; however, extra-nodal involvement has also been reported. Because a consistent standard therapy for IDCS has not been established to date, we report a case of the successful treatment of disseminated IDCS using ABVD chemotherapy (doxorubicin, bleomycin, vinblastine, and dacarbazine). A 64-year-old man was diagnosed with IDCS on the basis of immunohistochemical findings of a biopsy specimen of the inferior nasal concha. Immunohistochemical staining showed a positive reaction for CD68, leukocyte common antigen, and S-100 protein, but a negative reaction for CD34, CD1a, and CD21. Imaging studies showed cervical and axillary lymphadenopathies, subcutaneous nodules, and a soft tissue lesion in the nasal cavity. Treatment with the ABVD regimen resulted in complete remission after 8 cycles of chemotherapy.
Antigen-Presenting Cells ; Antigens, CD45 ; Biopsy ; Bleomycin ; Dacarbazine ; Dendritic Cell Sarcoma, Interdigitating ; Dendritic Cells ; Humans ; Middle Aged ; Nasal Cavity ; S100 Proteins ; Turbinates ; Vinblastine

Primary cardiac lymphoma (PCL) is an extremely rare and fatal neoplasm of the heart. Traditionally, it is defined as lymphoma involving the heart or pericardium. PCL has a poor prognosis because of the diagnostic difficulty and its location. We present the case of a 48-year-old man who presented with pericardial effusion and diffuse cardiac wall thickening. We first suspected infiltrative heart disease. However, even after performing a biopsy, we could not establish an accurate diagnosis. After 20 months, primary cardiac diffuse large B cell lymphoma (DLBCL) was diagnosed by cervical lymph node biopsy. In this case, after chemotherapy, the DLBCL lesions, including cardiac wall thickening, improved. The treatment outcome suggests that the diagnosis was diffuse infiltrative PCL with delayed extracardiac involvement.
Biopsy ; Diagnosis ; Drug Therapy ; Heart ; Heart Diseases ; Humans ; Lymph Nodes ; Lymphoma ; Lymphoma, B-Cell ; Middle Aged ; Pericardial Effusion ; Pericardium ; Prognosis ; Treatment Outcome

Successful hematopoietic stem cell transplantation (HSCT) involves the restoration of hematopoietic function after engraftment, arising from the differentiation and proliferation of hematopoietic stem cells. Several factors could influence the course of allogeneic-HSCT (allo-HSCT). Therefore, knowledge of serum proteome changes during the allo-HSCT period might increase the efficacy of diagnosis and disease prevention efforts. This study conducted proteomic analyses to find proteins that were significantly altered in response to allo-HSCT. Sera from five representative patients who underwent allo-HSCT were analyzed by 2-dimensional gel electrophoresis and liquid chromatography tandem mass spectrometry, and were measured on a weekly basis before and after allo-HSCT in additional 78 patients. Fourteen protein spots showing changes in expression were further examined, and most proteins were identified as acute phase proteins (APPs). Studies of 78 additional patients confirmed that C-reactive protein (CRP) and haptoglobin undergo expression changes during allo-HSCT and thus may have the potential to serve as representative markers of clinical events after allo-HSCT. Maximal CRP level affected the development of major transplant-related complications (MTCs) and other problems such as fever of unknown origin. Particularly, an increase in CRP level 21 days after allo-HSCT was found to be an independent risk factor for MTC. Maximal haptoglobin and haptoglobin level 14 days after allo-HSCT were predictive of relapses in underlying hematologic disease. Our results indicated that CRP and haptoglobin were significantly expressed during allo-HSCT, and suggest that their level can be monitored after allo-HSCT to assess the risks of early transplant-related complications and relapse.
Adolescent ; Adult ; Biological Markers ; C-Reactive Protein/*metabolism ; Female ; Haptoglobins/*metabolism ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans ; Male ; Middle Aged ; Proteome/*metabolism ; Proteomics ; Transplantation Conditioning ; Transplantation, Homologous ; Young Adult

BACKGROUND: High-dose melphalan (200 mg/m2) with autologous stem cell transplantation (ASCT) is the standard treatment for young patients with multiple myeloma (MM). However, the response rates after ASCT are often unsatisfactory. We performed a pilot study by using bortezomib-melphalan as conditioning regimen for ASCT in Korean patients with MM. METHODS: The conditioning regimen consisted of administration of intravenous infusion of bortezomib 1.0 mg/m2 on days -4 and -1 and melphalan 50 mg/m2 (day -4) and 150 mg/m2 (day -1). In this study, we enrolled 6 newly diagnosed patients and 2 patients with relapse. RESULTS: The disease status of the 6 newly diagnosed patients at ASCT was as follows: 1 complete remission (CR), 1 very good partial remission (VGPR), and 4 partial remissions (PRs). The disease status of the 2 relapsed patients at ASCT was PR. All patients except 1 showed adequate hematologic recovery after ASCT. The median time for the absolute neutrophil counts to increase over 500/mm3 was 13 days (range, 10-19 days). Six patients with VGPR or PR at the time of transplantation showed an improvement in response to CR after ASCT. The patients were followed up without any maintenance treatment after ASCT except 1 patient who died during ASCT. During the follow-up period, CR was maintained in 3 newly diagnosed patients, but the other 4 patients, including 2 newly diagnosed patients, relapsed. CONCLUSION: Conditioning regimen consisting of bortezomib and melphalan may be effective for ASCT in MM; however, the feasibility of this regimen should be further evaluated in large study populations.
Boronic Acids ; Follow-Up Studies ; Humans ; Infusions, Intravenous ; Melphalan ; Multiple Myeloma ; Neutrophils ; Pilot Projects ; Pyrazines ; Recurrence ; Stem Cell Transplantation ; Stem Cells ; Transplants ; Bortezomib

A variety of clonal cytogenetic abnormalities have been reported in aggressive natural killer (NK)-cell lymphoma and leukemia. Recent chromosomal microarray studies have shown both gain and loss of 1q and loss of 7p as recurrent abnormalities in aggressive NK-cell leukemia. Here, we report a case of aggressive NK-cell leukemia with complex chromosomal gains and losses, as confirmed by chromosomal microarray analysis. The patient showed an aggressive clinical course, which was complicated by hemophagocytic lymphohistiocytosis. Conventional cytogenetic analysis revealed trisomy 3 and 1q gain only. However, chromosomal microarray analysis detected an additional gain of 1q21.1–q24.2 and a loss of 1q24.2–q31.3. These abnormal lesions might play a role in the pathogenesis of aggressive NK-cell leukemia by inactivating tumor suppressor genes or by activating oncogenes. These results suggest that chromosomal microarray analysis may be used to provide further genetic information for patients with hematological malignancies, including aggressive NK-cell leukemia.
Chromosome Aberrations ; Cytogenetic Analysis ; Genes, Tumor Suppressor ; Hematologic Neoplasms ; Humans ; Leukemia ; Lymphohistiocytosis, Hemophagocytic ; Lymphoma ; Microarray Analysis ; Oncogenes ; Trisomy

Background/Aims: The prognosis of small cell lung cancer (SCLC) is still poor because of rapid recurrence, despite good response to initial chemotherapy. Additionally, patients’ old ages and comorbidities are often obstacles that make it difficult to apply subsequent treatment after initial treatment. This retrospective study analyzed the correlation of post-progression survival (PPS) with overall survival (OS), and prognostic factors including comorbidities to figure out impact of subsequent chemotherapy on OS in elderly extensive disease SCLC. Methods: We analyzed 101 patients of age 65 years or older who were recently diagnosed with extensive disease SCLC (ED-SCLC) in Korea University Medical Center between January 1995 and December 2015. The degree of comorbidity was scored using simplified comorbidity score (SCS). Correlation between PPS, progression-free survival (PFS) and OS was analyzed using a Pearson correlation coefficient. Cox proportional hazards regression was employed to examine the influence of clinical variables on survival. Results: Median age of patients was 71 years old (range, 65 to 83). Median OS was 8.7 months (range, 0.3 to 42.7). PPS was a reliable factor on OS than PFS (R2 = 0.852, p < 0.001). Prognostic factors associated with improved survival were SCS < 9, administration > 4 cycles of first line chemotherapy and subsequent second line chemotherapy. Conclusions PPS was more correlated with OS than PFS in elderly patients with ED-SCLC. The most important prognostic factors for PPS and OS included SCS and second line chemotherapy. Patients receiving subsequent treatment had increased OS regardless of degree of comorbidity.

BACKGROUND/AIMS: Despite several reports on clinical aspects of anemia and malignancy, little is known of male patients with iron-deficiency anemia (IDA) and malignancy in Korea. We examined the cause of anemia, prevalence of and factors associated with malignancy, and treatment response to iron therapy in male IDA patients. METHODS: The results of 202 males with IDA seen from March 2008 to June 2013 were analyzed retrospectively. The patients were divided into two groups based on the causes of anemia: the cancer group included patients with anemia caused by malignancy and the non-cancer group included patients with anemia due to other causes. We compared the clinical characteristics and response to iron therapy between the two groups. RESULTS: The most common cause of IDA was bleeding (42.6%). The prevalence of malignancy was 11.9%, with colorectal cancer (58.3%) being the most common. Among the cancer patients (n = 24), 22 patients (91.7%) were age 50 or older. Independent factors associated with malignancy were old age (OR, 1.05; p = 0.026) and a positive stool occult blood test (OR, 7.48; p = 0.001). The treatment response to iron therapy based on a normalized hemoglobin level was lower in the cancer group (OR, 0.49; p = 0.31), but the difference did not reach statistical significance. The treatment response based on the mean hemoglobin level was significantly lower in the cancer group (12.6 +/- 2.2 vs. 13.8 +/- 1.6 g/dL, p = 0.016). CONCLUSIONS: Old age and a positive stool occult blood test were independent risk factors for malignancy in male IDA patients. We recommend screening for malignancy in patients older than 50 years or with a positive stool occult blood test.
Anemia ; Anemia, Iron-Deficiency* ; Colorectal Neoplasms ; Hemorrhage ; Humans ; Iron ; Korea ; Male ; Mass Screening ; Occult Blood ; Prevalence* ; Retrospective Studies ; Risk Factors

Atypical chronic myeloid leukemia (aCML) is a rare leukemic disorder that shows myelodysplastic and myeloproliferative features simultaneously. The Janus kinase 2 gene V617F mutation (JAK2V617F) in aCML has been the source of much controversy. Some JAK2V617F positive cases have been reported but others observed no JAK2V617F mutation in aCML as defined by WHO classification. Recently, we experienced a case of aCML with JAK2V617F mutation with typical myelodysplastic/myeloproliferative features in peripheral blood and bone marrow aspirates. The karyotype was normal and no BCR/ABL1, PDGFRA or PDGFRB gene rearrangement was noted with FISH analysis. JAK2V617F mutation of the case was identified with amplification refractory mutation system PCR and direct sequencing. We also studied JAK2V617F mutation status in 3 additional cases of previously diagnosed aCML in our institution, but no mutation was identified.
Bone Marrow ; Gene Rearrangement ; Janus Kinase 2 ; Karyotype ; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative ; Myelodysplastic Syndromes ; Myeloproliferative Disorders ; Polymerase Chain Reaction ; Receptor, Platelet-Derived Growth Factor beta