POEMS Syndrome: Bone Marrow, Laboratory, and Clinical Findings in 24 Korean Patients

POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.

A Case of Type II Enteropathy-Associated T-Cell Lymphoma with Epstein-Barr Virus Positivity

No abstract available.

Clinical characteristics, pathological distribution, and prognostic factors in non-Hodgkin lymphoma of Waldeyer's ring: nationwide Korean study

BACKGROUND/AIMS: In Asia, the incidence of non-Hodgkin lymphoma (NHL) has increased in recent decades. Waldeyer's ring (WR) is the most common site of NHL involving the head and neck. In this study, the pathological distribution of WR-NHL and its clinical features were analyzed retrospectively. METHODS: From January 2000 through December 2010, we analyzed the medical records of 328 patients from nine Korean institutions who were diagnosed with WR-NHL. RESULTS: The study group comprised 197 male and 131 female patients with a median age of 58 years (range, 14 to 89). The rate of localized disease (stage I/II) was 64.9%, and that of low-risk disease (low/low-intermediate, as defined by the International Prognostic Index) was 76.8%. Diffuse large B-cell lymphoma (DLBCL; 240 patients, 73.2%) was the most common pathologic subtype, followed by peripheral T-cell lymphoma (14 patients, 4.3%) and nasal NK/T-cell lymphoma (14 patients, 4.3%). WR-NHL occurred most frequently in the tonsils (199 patients, 60.6%). Extranodal involvement was greater with the T-cell subtype (20 patients, 42.5%) compared with the B-cell subtype (69 patients, 24.5%). Multivariate analyses showed that age > or = 62 years, T-cell subtype, and failure to achieve complete remission were significant risk factors for overall survival. CONCLUSIONS: DLBCL was found to have a higher incidence in Korea than those incidences reported by other WR-NHL studies. T-cell lymphoma occurred more frequently than did follicular lymphoma. T-cell subtype, age > or = 62 years, and complete remission failure after first-line treatment were significant poor prognostic factors for overall survival according to the multivariate analysis.

Langerhans Cell Histiocytosis Followed by Hodgkin's Lymphoma

A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma.

Desmoplastic Small Round Cell Tumor Achieved Complete Remission after High-Dose Chemotherapy with Aggressive Local Treatment Modalities / 대한내과학회지

A desmoplastic small-round-cell tumor (DSRCT) is a rare, aggressive neoplasm that develops mostly in the abdominal cavity in children and young adults. We present a case of a 19-year-old male with right upper quadrant discomfort for 3 months. On abdominal computerized tomography, multiple huge and demarcated masses were found in the liver, retroperitoneal lymph nodes, and peritoneal and retroperitoneal cavities. Fine needle aspiration biopsy of the hepatic mass was performed and DSRCT was diagnosed by hematoxylin and eosin staining and immunohistochemical analysis. He was treated initially with high-dose systemic chemotherapy (alternating schedules of cyclophosphamide, vincristine, doxorubicin, ifosfamide, and etoposide), underwent two debulking surgeries and pelvic irradiation between systemic chemotherapy schedules, and achieved complete remission after the 15 months of treatment duration. We report this case to emphasize the importance of aggressive local treatment modalities as well as high-dose systemic chemotherapy for treatment of DSRCT even with initially unresectable or extensively metastatic presentation.

A Case of CD5 Negative Chronic Lymphocytic Leukemia

Neoplastic lymphoid cells of chronic lymphocytic leukemia (CLL) typically co-express CD5 and CD23. CD5-negative CLL is a rare variant of CLL; only 1 case of it has been reported in Korea. We describe a case of CD5-negative CLL. A 48-year-old female complained of a palpable neck mass that had been present for over 1 year. The initial WBC count was 7,300/microliter, with 69% lymphocytes. A CT scan revealed multiple enlarged lymph nodes, both of each in the neck, axilla, and common iliac areas. The athologic results of the cervical lymph node was consistent with small lymphocytic lymphoma, of which tumor cells do not express CD5. In a bone marrow study, neoplastic lymphoid cells comprise 34.8% of all nucleated cells, which showed small size, round nuclei with clumped chromatin, and sparse cytoplasm. Immunophenotyping of small lymphoid cells displayed phenotypes that were CD45-, CD23-, CD20-, and CD19-positive, but CD5-negative. The patient was diagnosed with CD5-negative CLL, and has been followed up for 2.5 years after chemotherapy.

Laparoscopic Splenectomy for Patients with Idiopathic Thrombocytopenic Purpura / 대한소화기내시경학회지

The success and rapid spread of laparoscopic cholecystectomy have prompt the application of laparoscopic approach to abdominal surgical problems. As better in- strumentation is introduced, more complicated surgical procedrues will be performed. In some hematologic disorders, splenectomy was required to cope with these disorders. So laparoscopic splenectomy also has been perfomed inspite of several specific difficulties for handling and mobilization of a parenchymatous organ and the retrieval of the specimen. We experienced a case of idiopathic thrombocytopenic purpura which was treated safely and successfully by laparoscopic splenectomy in 35-year-old female. This technique allows the patient to return to normal activity sooner than with open splenectomy.

Isotretinoin and granulocyte colony stimulating factor induced complete remission in a patient with acute promyelocytic leukemia complicated by life threatening wound infection / 대한혈액학회지

No abstract available.

The Efficacy and Safety of DA-3030 (Recombinant Human Granulocyte Colony-Stimulating Factor) in Neutropenia after the Remission Induction Chemotherapy in Patients with Acute Myelogenous Leukemia / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: This study was conducted to determine the efficacy and safety of DA-3030 (a recombinant methionyl human granulocyte colony-stimulating factor, rhG-CSF), after remission induction chemotherapy, in patients with acute myelogenous leukemia (AML). MATERIALS AND METHODS: After the remission induction chemotherapy, with idarubicin (12 mg/m2/day for 3 days) and cytarabine (200 mg/m2/day for 7 days), 26 patients with newly diagnosed AML were assigned to receive DA-3030 (200mug/m2/day), starting 24 hours after the completion of the remission induction chemotherapy, until their neutrophil count recovered to greater than 1, 000/muL for 3 consecutive days. RESULTS: The median time from the initiation of the chemotherapy to the neutrophil recovery of 1, 000/muL was 21 days (range, 12~41). Treatment with DA-3030 was not associated with significant adverse side effects. The most frequently reported side effects were musculo-skeletal pain (13%) and headache (13%). CONCLUSION: The DA-3030 is a safe rhG-CSF for the treatment of neutropenia after remission induction chemotherapy in patients with AML.

Ondansetron compared with ondansetron plus metoclopramide in the prevention of cisplatin-induced emesis

To determine the contribution of metoclopramide to the efficacy of ondansetron in control of cisplatin-induced emesis, ondansetron was compared with ondansetron plus metoclopramide for antiemetic efficacy in a randomized double-blind trial. Enrolled 66 patients were treated with cisplatin(60mg/m2) in combination with etoposide, flourouracil, or vinblastine, and randomized to receive either ondansetron alone or ondansetron plus metoclopramide. Sixty patients were evaluable. Complete or major control of acute emesis was achieved in 96.6% (29/30) of patients given ondansetron plus metoclopramide and in 80% (24/30) receiving ondansetron alone, with no statistical significance (P = 0.07). However, delayed emesis (days 2-6) was better controlled by combination therapy than by ondansetron alone with 22 of 30 (73.4%) and 11 of 30 (36.7%), respectively (P = 0.03). No major drug-related side effects were observed. These results suggest that ondansetron plus metoclopramide is superior to ondansetron alone in the control of cisplatin induced delayed emesis without significant side effects.

Usefulness of IMI and HPC of Sysmex SE-9000, and IRF and HFR of Sysmex R-3000 to Refine Apheresis Timing of Peripheral Blood Stem Cell Harvest / 대한혈액학회지

BACKGROUND: The Sysmex SE-9000 and R- 3000 automated cell counters provide estimates of immature cells referred to as immature myeloid information (IMI), hematopoietic progenitor cells (HPC), immature reticulocyte fraction (IRF) as high and medium fluorescent reticulocytes, and high fluorescence ratio (HFR) as high fluorescent reticulocytes. The aim of this study was to evaluate whether these parameters were useful to refine apheresis timing of peripheral blood stem cell (PBSC) harvest. METHOD: For 140 peripheral blood harvest procedures of 26 patients, pre-harvest peripheral blood (PB) WBC, mononuclear cells (MNC), IMI, HPC, CD34-positive cells, reticulocyte (%, number), IRF and HFR were tested and compared with harvested CD34-positive cell content. RESULTS: Correlation coefficients between pre-harvest WBC, MNC, IMI, HPC, CD34-positive cells, reticulocyte %, reticulocyte number, IRF and HFR of PB and harvested CD34-positive cell content were 0.15, 0.06, 0.60, 0.78, 0.77, 0.004, 0.06, 0.28 and 0.40. Applying the criteria IMI 300X10degrees/L, HPC 5X10degrees/L and CD34-positive cells 5X10derees/L of PB on the first day of 30 cycles of harvests, positive predictive value to predict the mean CD34+ cell count over 0.5X106/kg per one leukapheresis and negative predictive value to predict the mean CD34+ cell count less than 0.5X10derees/kg per one leukapheresis were 73.3%/93.3%, 57.8%/90.9% and 78.6%/ 93.7% respectively. CONCLUSION: Pre-harvest PB IMI and HPC of Sysmex SE-9000 are comparable with PB CD34- positive cells in terms of correlation with harvested CD34-positive cell content. For PB IMI and HPC are simple, inexpensive and rapid to get results, PB IMI and HPC are useful to refine apheresis timing of PBSC harvests and to screen poor-mobilizers.

Acute promyelocytic leukemia is a distinct subset of acute myelocytic leukemia with unique clinicopathologic characteristics including longer duration of relapse free survival: experience in 13 cases

Acute promyelocytic leukemia(APL) is a subtype of acute myelocytic leukemia(AML) associated with unique features such as the presence of atypical promyelocytes and bleeding tendency due to disseminated intravascular coagulation(DIC). In a retrospective study, we analyzed 96 cases of AML seen at our hospital between June, 1989 and December 1993. Thirteen cases of APL(14%) were identified and their clinicopathologic characteristics were analyzed. The 86 cases of other types of AML served as controls. The distinct clinicopathologic features of APL as contrasted to other types of AML included younger age of patients, shorter duration of symptom before diagnosis, higher level of albumin at presentation, and a higher proportion of patients having coagulation abnormalities (75 vs. 25%). Bone marrow cellularity was higher in APL when compared to other types of AML (100 vs. 90%, P = 0.013). Of 13 patients with APL, 4 died of bleeding/sepsis between days 2 to 4 after admission. Seven of 9 patients who received induction therapy achieved complete remission(CR). CR rate in APL was similar to other types of AML (78 vs. 64%, P = 0.743). Five of seven patients who achieved CR remain in continuous CR at 9+ to 42+ months. CR duration is significantly longer in APL when compared to other types of AML (P = 0.029). In conclusion, this study showed that APL is a distinct entity among subtypes of AML with clinically significant bleeding tendency and rapidly fatal course if untreated. With appropriate antileukemic therapy, CR can be achieved in the majority of patients and the patients show a longer duration of CR when compared to other types of AML.

A Case of Treatment-Related Myelodysplastic syndrome and Acute Myelogenous Leukemia Following High-Dose Chemotherapy with Autologous Stem Cell Transplantation for Non-Hodgkin's Lymphoma

Treatment-related myelodysplastic syndrome (t-MDS) and acute myelogenous leukemia (t-AML) are now well established as complications of cytotoxic chemotherapy. We experienced a 28-yr-old female patient who developed t-MDS/t-AML with characteristic chromosomal abnormalities including 11q23 chromosomal rearrangement following high-dose chemotherapy with autologous stem cell transplantation (ASCT) for non-Hodgkin's lymphoma. The patient was admitted with bulky abdominal masses of B cell lineage non-Hodgkin's lymphoma. After 2 cycles of systemic chemotherapy of the Vanderbilt regimen, the patient underwent ASCT with high dose chemotherapy of the BEAC regimen. She also received radiation of 48 Gy for the residual periportal lymphadenopathy. The initial cytogenetic analysis of the infused mononuclear cells revealed a normal karyotype. Twenty two months after the ASCT, pancytopenia was noted and her bone marrow aspirate showed dysplastic hemopoiesis with myeloblasts up to 12% of nonerythroid nucleated cells. The patient was diagnosed as t-MDS (refractory anemia with an excess of blasts). Cytogenetic analysis showed complex chromosomal abnormalities including 11q23 rearrangement, which is frequently found in topoisomerase II inhibitor-related hematologic malignancies. Four months later, it was noted that the t-MDS had evolved into an overt t-AML. Cytogenetic analysis showed an evolving pattern with more complex abnormalities. The patient was treated with combination che-motherapy, but her leukemic cells were resistant to the therapy.

A Case of Placental Metastasis from Advanced Gastric Carcinoma / 대한암학회지

Placental and fetal involvement by matenal malignancy is rare. We report a case of placental metastasis from advanced gastric carcinoma in a 27 year-old woman. The patient also had disseminated bone metastasis, bone marrow involvement, malignant ascites, multiple lymphadenopathy, and disseminated intravascular coagulopathy. Cut surface of the placental body showed many, variable-sized, grayish white nodules and plaques. Light microscopic finding showed sheets of poorly differentiated adenocarcinoma in intervillous spaces. Villi were not invaded. Despite palliative chemotherapy the patient died of massive gastric cancer bleeding. But the patients child is alive and doing well with age of 11 months. We suggest that the presence of malignancy in pregnancy demands complete evaluation of the placenta and adequate follow-up of the infant for the sign of involvement.

Clinical Features of Neuroendocrine Lung Cancer / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: This study was performed to investigate the clinical features of neuroendocrine lung cancer. MATERIALS AND METHODS: We performed a retrospective review of the histopathology and clinical information of 21 patients diagnosed as having neuroendocrine lung cancer between 1995 and 1999. RESULTS: Nineteen cases were male and 2 were female. The median age was 64 years (range: 45~80). Pathologic classification were atypical carcinoid (AC) in 2 cases, large cell neuroendocrine carcinoma (LCNEC) in 7 cases, and intermediate cell neuroendocrine carcinoma (ICNC) in 12 cases. Nine patients received tumor resection as first line therapy; adjuvant chemotherapy was given to 3 patients. Concurrent chemoradiotherapy was given to 1 patient. Six patients received palliative chemotherapy. The chemotherapy regimen included etoposide cisplatin in 5 cases and vinorelbine+cisplatin in 1 case. The median survival times were 11, 16 and 59 weeks for AC, LCNEC and ICNC, respectively. The estimated 2-year survival rates were AC 0%, LCNEC 22% and ICNC 31%. CONCLUSION: Surgery may have a positive effect on survival in patients with early stage cansers. Further investigation is required to improve survival in cases of advanced stage cancer.

Myelodysplastic syndrome that progressed to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormality: a case report

Myelodysplastic syndrome is a closely related group of acquired bone marrow disorders characterized by ineffective and dysplastic hematopoiesis. These clonal disorders frequently progress to acute leukemia. Acute myelomonocytic leukemia with eosinophilia is characterized by an increase in abnormal eosinophils in the bone marrow, relatively good clinical course and inv (16) chromosomal abnormality. We experienced one case of refractory anemia with excess blasts which progressed to refractory anemia with excess blasts in transformation and finally to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormalities of der (1;7).

Prognostic factors of acute myelocytic leukemia: an analysis of 132 patients in a single institution

Patients with acute myelocytic leukemia (AML) have varied outlooks for survival after the diagnosis. To identify pretreatment prognostic indicators in AML, we analyzed 132 cases of AML seen at our hospital between June, 1989 and December, 1994. The median age of the patients was 40 years (range, 15-81). There were 63 male and 69 female patients. One hundred eight patients (82%) received induction chemotherapy which was based on cytarabine plus anthracyclines. Sixty six patients achieved complete remission (CR) and the CR rate among the patients given induction chemotherapy was 61%. The median duration of CR was 11.2 months. After median follow up of 6.6 months (range 0.5-51.4), 26 patients (39%) remain in continuous CR. The median duration of overall survival of the patients was 6.7 months. After median follow up of 10.6 months (range, 0.1-52.7), 41 patients (31%) are alive. Variables affecting duration of CR included the age of the patients, performance status of the patients, percentage of blast in the peripheral blood, hemoglobin level, percentage of blast in the bone marrow, FAB subtype, and CD7 marker positivity. Variables affecting survival duration included age of the patients, performance status of the patients, absolute blast count (ABC) in the peripheral blood, bone marrow cellularity, the percentage of blast in the bone marrow, and CD5 marker positivity. Multivariate analysis showed that the age of the patients and percentage of blast in the bone marrow were significant independent indicators for overall survival of the patients. Further studies utilizing cytogenetics and molecular characteristics of leukemic cell are warranted to better define the prognostic factors of patients with AML.

Results of Treatment and Prognostic Factors of Lymphoblastic Leukemia: An Analysis in 33 Patients / 대한내과학회지

OBJECTIVES: Rate of complete remission and long-term survival in adult acute lymphoblastic leukemia group has not been as satisfactory as that in childhood ALL. Recently introduction of induction chemotherapy of more intensive combination and various trials of postremission therapy are making improved results better looked forward to. And subtypes of ALL according to the degree of differentiation into T and B cells are identified by using immunologic markers hopefully to work out proper treatment for each subtype. METHODS: We analited results of treatment and differences of complete remission rate, remission duration and overall survival as to various immunologic markers and clinicopathologic characteristics in 33adult ALL patients. RESULTS: Eighty five percents of the 27cases that had VPDL chemotherapy achieved complete remission and both overall median survival and mediom duration of remission were 52weeks. No definite prognostic factors were detected influencing complete remission rate, remission duration and overall survival except that patients with serum albumin level higher than 4.0mg/dL showed highter complete remission rate. Although mature B-ALL showed the shortest overall median survival, degree of differenciation of B-cell and other immunologic markers did not influence on complete remission rate, remission duration or overall survival. CONCLUSION: Further studies are needed to delire the prognostic factors in adult ALL.

Hyperfractionated Radiotherapy Following Induction Chemotherapy for Stage III Non-Small Cell Lung Cancer: Random iced for Adjuvant Chemotherapy vs. Observation / 대한치료방사선과학회지

Since Jan. 1991 a prospective randomized study for Stage III unresectable non small cell lung cancer(NSCLC) has been conducted to evaluate the response rate and tolerance of induction chemotherapy with MVP followed by hyperfractionated radiotherapy and evaluate the efficacy of maintenance chemotherapy in Asan Medical Center. All patients in this study were treated with hypefractionated radiotherapy (120 cGy/fx BID, 0480 cGy/54 fx) following 3 cycles of induction chemotherapy, MVP (Mitomycin C 6 mg/m2, Vinblastin B mg/ m2, Cisplatin 60 Mg/ m2) and then the partial and complete responders from induction chemotherapy were randomized to 3 cycles of adjuvant MVP chemotherapy group and observation group. 48 patients were registered to this study until December 1992; among 48 patients 3 refused further treatment after induction chemotherapy and 6 received incomplete radiation therapy because of patient's refusal, 39 completed planned therapy. Twenty-three(58%) patients including 2 complete responders showed response from induction chemotherapy. Among the 21 patients who achieved a partial response after induction chemotherapy, 1 patient rendered complete clearance of disease and 10 patients showed further regression of tumor following hypefractionated radiotherapy. Remaining 10 patients showed stable disease or progression after radiotherapy. Of the sixteen patients judged to have stable disease or progression after induction chemotherapy, seven showed more than partial remission after radiotherapy but nine showed no response in spite of radiotherapy. Of the 35 patients who completed induction chemotherapy and radiotherapy, 25 patients(64%) including 3 complete responders showed more than partial remission. Nineteen patients were randomized after radiotherapy. Nine patients were allocated to adjuvant chemotherapy group and 4/9 shewed further regression of tumor after adjuvant chemotherapy. For the time being, there is no suggestion of a difference between the adjuvant chemotherapy group and observation group in distant metastasis rate and survival. Median survival time was 13 months. Actuarial survival rates at 6, 12 and 18 months of 39 patients who completed this study were 84.6%, 53.7% and 40.3%, respectively. The partial and complete responders from induction chemotherapy showed significantly bettor survival than non-responders(p=0.028). Incidence of radiation pneumonitis in this stuffy group was less than that in historical control group inspite of induction chemotherapy. All patients tolerated hyperfractionated radiotherapy without definite increase of acute complications compared with conventional radiotherapy group. The longer fellow up is needed to evaluate the efficacies of induction and maintenance chemotherapy and survival advantage by hypefractionated radiotherapy but authors are encouraged with an excellent tolerance, higher response rate and improvement of one year survival rate in patients of this study.

A Case of a Young Woman with Hepatosplenic gamadelta T-cell Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

Most T-cell lymphomas arise from mature alpabeta T-cells and commonly involve the nodes. Lymphomas bearing the gamadelta T-cell receptor (TCR) are very rare, and involve the lymph nodes minimally, if at all. Hepatosplenic gamadelta T-cell lymphoma is a recently identified, rare entity in which lymphoma cells bearing the gamadelta TCR infiltrate the sinusoids of the liver, splenic red pulp, and bone marrow. Its leukemic transformation is even more rare. Recently, we experienced a case of hepatosplenic gamadelta T-cell lymphoma in a 19-year-old woman who presented with epigastric pain, fever, massive splenomegaly, andpancytopenia. The splenectomy specimen and excisional biopsy of the liver revealed the infiltration of atypical T lymphocytes with the immunophenotypic markers of CD3 (+), CD45RO (pan-T antigen) (+), TIA-1(+), CD4(-),CD8 (-), CD56 (-), and S100 (-) in the sinusoids of the liver and splenic red pulp. Polymerase chain reaction (PCR) showed that these cells had the expression of the TCR gama gene rearrangements. Though the pancytopenia had improved after the splenectomy, the response of chemotherapy was transient. Her disease progressed rapidly and she expired in the leukemic phase. We report a case of hepatosplenic gamadelta T-cell lymphoma that developed in a young woman, along with a brief review of the literature.