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.

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.

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

No abstract available.

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.

Primary Intestinal Lymphoma

PURPOSE: Primary gastrointestinal lymphoma is the most common form of extranodal lymphoma. The clinical features, histological distributions, treatment results and prognosis of the primary intestinal lymphoma were evaluated. METHODS: A retrospective study was performed on 62 patients with primary intestinal lymphoma, as defined by Lewin's criteria, from May 1990 to February 2002. The WHO classification and Ann Arbor staging system were used for histological classification and staging, respectively. RESULTS: The sex ratio of the patients was 43: 19 (male: female), and the median age was 54 years. Abdominal pain, a palpable mass, and bleeding were the most frequent symptoms on presentation. The ileocecal area was the most frequent pathological site. Fifty-three cases were non- Hodgkin's lymphoma of B-cell origination; all of the remaining were T-cell originated. The mean survival period of B-cell and T-cell originated were 59.3 and 14.3 months, respectively (P<0.05). The 5 year survival rates of the patients in stage IE and IIE, and stage IIIE and IVE, were 52.4 and 32.6%, respectively (P=0.03). Six patients received surgery, 17 chemotherapy, and 39 surgery with adjuvant chemotherapy. Among the patients confined to stage IE and IIE, the 3 year survival rates of the surgery and surgery with adjuvant chemotherapy groups were 34 and 84%, respectively (P=0.0049). CONCLUSION: Primary gastrointestinal lymphoma of B-cell origination was predominant in relation to the WHO classification and revealed a better prognosis when compared to the T-cell originated lymphoma. For the patients with localized intestinal lymphoma, multimodality treatment (surgery with adjuvant chemotherapy) is preferred to the sole administration of chemotherapy.

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.

The Efficacy of High-dose Melphalan with Autologous Peripheral Blood Stem Cell Transplantation in Patients with Multiple Myeloma

Although high-dose therapy (HDT) with autologous hematopoietic stem cell transplantation (ASCT) is widely accepted as an effective and safe consolidation therapy for multiple myeloma (MM), few reports on its efficacy are available in Korea. We present the results of a prospective phase II study, involving 33 patients with MM treated with HDT with ASCT. The treatment consisted of 4 courses of VAD (vincristine, adriamycin, dexamethasone) induction, peripheral blood stem cell collection, and high-dose melphalan with stem cell infusion. The overall response rate was 93%, with 45% of patients having complete responses. The toxicity was predictable and tolerable. With a median follow-up of 27.6 months, the 2-year event free survival rate was 43%. At the time of writing, the median overall survival duration had not been reached with 2-year survival and projected 3-year survival rates of 81% and 74%, respectively. The overall survival was significantly better than that of the historical control patients (N=82) treated with conventional chemotherapy at our institution. The results suggest that HDT with ASCT is a valuable first or second-line treatment for patients with MM.

Primary Orbital Lymphoma: A Retrospective Analysis of Results of Radiation Therapy / 대한방사선종양학회지

PURPOSE: This study evaluated the treatment outcomes, patterns of failure, and treatment related complications of primary lymphoma patients who received definitive radiation therapy. MATERIALS AND METHODS: A retrospective analysis was undertaken for 31 patients with primary orbital lymphoma at the Asan Medical Center between February 1991 and April 2001. There were 18 males and 13 females with ages ranging from 3 to 73 years (median, 44 years). The involved sites were 9 conjunctivae, 12 eyelids and 10 other orbits. The histological types were 28 MALT lymphomas (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type), 1 diffuse large B-cell lymphoma, 1 anaplastic large cell lymphoma and 1 lymphoblastic lymphoma. The Ann Arbor stages were all IE (100%). Ann Arbor stage III or IV patients were excluded from this study. Bilateral orbital involvement occurred in 6 cases. Radiation therapy was given with one anterior port of high energy electrons (6~16 MeV) for the lesions located at the anterior structures like the conjunctivae or eyelids. Lesions with a posterior extension or other orbital lesions were treated with 4 or 6 MeV photons with appropriately arranged portals. In particular, lens blocks composed of lead alloy were used in conjunctival or eyelid lesions. Twelve patients received chemotherapy. The median follow-up period was 53 months. RESULTS: The 5-year overall, cause-specific, and disease-free survival was 91%, 96%, and 80%, respectively. The complete response rate 6 months after radiation therapy was 100%. Local recurrences were observed in 2 patients at 16 and 18 months after completion of radiation treatment. They were salvaged with additional radiation therapy. Two patients developed distant metastases. A MALT lymphoma patient with a lung relapse was successfully salvaged with radiotherapy, but the other lymphoblastic lymphoma patient with bone marrow relapse expired. There were no severe complications but 5 patients developed radiation-induced cataracts and 2 patients developed dry eye. CONCLUSION: Most primary orbital lymphomas consisted of MALT lymphomas. Radiation therapy was a successful treatment modality for orbital lymphoma without any severe complications. In cases of local relapses, radiation therapy is also a very successful salvage treatment modality.

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.

Preliminary Results of Paclitaxel, Cisplatin and Concurrent High-Dose Radiation Therapy for Locally Advanced Non-Small-Cell Lung Cancer / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: To investigate the feasibility, toxicity and response rate, of concurrent chemoradiation therapy with paclitaxel/cisplatin in stage III locally advanced non-small cell lung cancer (NSCLC). MATERIALS AND METHODS: Between May 1999 and December 2000, 80 patients with stage III NSCLC were enrolled in a prospective protocol. Radiotherapy was given to a total dose of 70.2 Gy (daily fraction of 1.8 Gy for 5 days), over an 8 week period, on the gross tumor volume, combined with chemotherapy. The concurrent chemotherapy consisted of paclitaxel (40 mg/m2) and 20 mg/m2 cisplatin per week for 8 consecutive weeks. All patients received 3-D conformal radiotherapy using CT-simulated planning. Acute toxicities were evaluated by the RTOG scale. The median follow-up period was 16 months, ranging from 3 to 29 months. RESULTS: Of the 80 patients, 71 received treatment per protocol, with minor variation of protocol delivery. The median age of the patients was 60 years. Karnofsky Performance status were 100 and 90 in 62 patients, and 80 and 70 in 9, respectively. Weight loss of less than 5% for 6 months was observed in 22 patients. The response to treatment was evaluated from the radiological findings. Complete and partial responses were observed in 8 and 51 patients, respectively. Ultimately, 82% of patients (included complete responses: 8 cases) obtained more than a partial response. Although, radiation induced esophagitis was the most common treatment related toxicity, occurring in 44 patients (69%), severe radiation esophagitis like, grade 3, was observed in only 3 patients, and the most acute toxicities had completely recovered 1 month following treatment. The overall 2-year actuarial and progression free survivals were 56 and 45%, respectively. CONCLUSION: This combined modality has activity with manageable toxicity and 23 months in mean survival time in patients with stage III NSCLC. A longer follow up will be required to realise the expected higher survival of these results.

Concurrent Etoposide/Cisplatin Combination Chemotherapy (EP) and Thoracic Radiotherapy after Two Cycles of EP for Limited Stage Small Cell Lung Cancer / Journal of the Korean Cancer Association, 대한암학회지

Purposes: Although the standard management of limited stage small cell lung cancer is concurrent platinum-based chemotherapy with thoracic radiotherapy (TRT), the optimal timing of the TRT remains controversial. We investigated the feasibility of concurrent chemoradiation for the patients with limited stage small cell lung cancer after 2 cycles of combination chemotherapy with Etoposide/Cisplatin (EP). MATERIALS AND METHODS: EP consisted of Etoposide 100 mg/m2 on day 1 to 3 and Cisplatin 70 mg/m2 on day 1. Six cycles were given to the responders every 4 weeks. Total 55 Gy (1.8 Gy once-daily or 1.2 Gy twice-daily, 5 days per week) of TRT were given to the patients who showed at least a partial response after 2 cycles of EP. The other patients were treated by the physician's decision. The patients with complete remission were recommended to receive prophylactic cranial irradiation. RESULTS: Fifty patients were enrolled. Thirty-five (70%) of them showed responses (2 complete remissions and 33 partial remissions) after 2 cycles of EP. Thirty-three of the responders were given TRT starting with the 3rd cycle of EP. The nonresponders were treated with salvage chemotherapy and TRT. After completion of treatment for 50 patients, the overall response rate was 86% (29 complete remissions, 14 partial remissions). One patient (2%) showed stable disease, and 6 (12%) showed a progressive disease. The median progression free survival was 326 days and the median survival time was 410 days. One-, 2-, 3-, 4- and 5-year survival rates were 62%, 24%, 14%, 9% and 6%, respectively. As hematologic toxicities during chemoradiation, 35.1% with grade III/IV neutropenia and 18.9% with grade III/IV thrombocytopenia were noted. Grade II/III radiation pneumonitis and radiation esophagitis were noted in 5/1 and 13/1 patients (15.2%/ 3.0% and 39.4%/3.0%), respectively. One patient died of septicemia during chemoradiation. CONCLUSION: The concurrent EP and TRT after 2 cycles of EP was feasible in limited stage small cell lung cancer. Further study is required for the indentification of optimum timing of TRT during combination chemotherapy.

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.

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.

Clinical Impacts of Tumor Cell Contamination of Hematopoietic Stem Cell Products in Metastatic Breast Cancer Patients undergoing Autologous Peripheral Blood Stem Cell Transplantation: Multicenter Trial

To determine whether the tumor cell contamination of peripheral blood stem cells influences clinical impacts on high-dose chemotherapy in patients with metastatic breast cancer, we analyzed carcinoembryonic antigen (CEA) mRNA in the apheresis products by nested RT-PCR (reverse transcriptase-polymerase chain reaction). A total of 38 metastatic breast cancer patients and ten normal healthy subjects as a negative control were included. Twenty out of 38 (51.3%) apheresis products from patients with metastatic breast cancer were positive for CEA mRNA. CEA mRNA was noted in 54.8% (17/31) of patients mobilized with chemotherapy plus G-CSF and 42.8% (3/7) of patients with G-CSF alone. There was no significant difference in age, estrogen receptor, menopausal status, mobilization method, disease free interval, or number of metastasis sites (1 vs >/=2) between positive and negative groups. The presence of CEA mRNA in apheresis products did not influence the time to progression and overall survival in both groups. However, both the univariate and the multivariate analysis disclosed that the number of metastasis was associated with survival significantly. We suggest that the tumor cell contamination does not predict poor treatment outcome in patients with metastatic breast cancer.

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.

Prognostic factors affecting response to chemotherapy and survival duration in Korean patients with multiple myeloma / 대한내과학회지

BACKGROUND: New therapeutic modalities such as high dose chemotherapy and stem cell support have been tried to prolong the survival period of the patients with multiple myeloma. However, little is known about the criteria for the application of those new therapies. There are only a few reports for the prognostic factors of multiple myeloma in Korea. The purpose of this study is to analyze the prognostic factors affecting chemotherapy response and survival in patients with multiple myeloma. METHODS: We retrospectively analysed the clinical records of 122 patients who were newly diagnosed as multiple myeloma by SWOG criteria, between November, 1989 and April, 1997 at Asan Medical Center. RESULTS: 1) The peak incidence was the 7th decade and male to female ratio was 1.3:1. The most common presenting symptom at first diagnosis was bone pain. 2) Initial clinical stage was as followed: stage I in 17.2% , stage II in 16.4% and 66.4% in III. The immunoglobulin classes were IgG in 51.6%, light chain only in 25.4%, IgA in 16.4%, IgD in 4.1%, and non-secretory type in 2.5%. Plasma cell types in bone marrow were classified as plasmablastic type in 45.9%, plasmacytic type in 54.1%. 3) Eighty two patients who recieved chemotherapy more than 3 cycles were evaluable for chemotherapy response. Overall response rate was 69.5%. Factors affecting response to chemotherapy were serum creatinine level, plasma cell type, total plasma cell percentage and plasmablast percentasge of total nucleated cells in bone marrow. 4) For total 122 patients, overall median survival period was 21 months, and estimated 5 year survival rate was 23.5%. Factors affecting survival were serum creatinine, corrected calcium, albumin, beta2-microglobulin level, response to chemotherapy, total plasma cell percentage and plasmablast percentage in bone marrow. CONCLUSION: Bone marrow findings at initial diagnosis are significantly associated with response to chemotherapy and survival duration.

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).

Clinical Courses and Prognostic Factors in Small Cell Lung Cancer / 대한내과학회지

BACKGROUND: Although small cell lung cancer is a chemosensitive disease, it grows rapidly and relapses frequently. Even with optimum treatment, only small portion of patients have experienced long-term survival. The objective of this study was to describe the clinical characteristics and therapeutic features, and to analyze the prognosis in small cell lung cancer. METHODS: We analyzed retrospectively 151 evaluable patients with histologically confirmed small cell lung cancer from August 1989 to June 1995 at our institution. Of 151 patients, 3 had surgery and chemotherapy, 59 had chemotherapy and chest irradiation, and 89 had chemotherapy only. RESULTS: Most patients(82.1%) were men, and the median age was 62 years. Of all patients, 49% had performance status of 0,1 and 59.6% had limited disease. The overall response rate was 67.8% : complete response 23.8%, partial response 44.4%. Complete responses were documented in all of three patients who had surgery and chemotherapy, 49.2% of those who had chemotherapy and radiotherapy, and 4.5% of those who had chemotherapy only. The median follow-up duration was 309 days. The median progression-free survival and overall survival were 256 days and 354 days, respectively: patients who had surgery and chemotherapy were 1631 days and 1631 days, those who had chemotherapy and radiotherapy were 344 days and 450 days, and those who had chemotherapy only were 186 days and 278 days, respectively; complete responders were 580 days and 710 days, partial responders were 231 days and 364 days, non-responders were 132 days and 151 days, respectively. Of 151 patients, 11.3% survived more than two years(long-term survival). Most long-term survivors had limited disease(82.4%) and good performance(76.5%). Long-term survival occurred in two patients of those who had surgery and chemotherapy, 16.9% of those who had chemotherapy and radiotherapy, and 5.6% of those who had chemotherapy only. Most long-term survivors(70.6%) had complete response. Twenty of 36 complete responders and 8 of 17 long-term survivors had disease relapses or progressions. Patients with limited disease, those with good performance, and those with normal alkaline phosphatase had a significantly higher complete response rate and longer progression-free survival and overall survival than their counterparts. Of pretreatment characteristics, stage and performance status were correlated complete response and survival, independently. complete response outcome was significant independent variable for survival. CONCLUSION: The disappointing results in this disease support the need for both new treatment strategies to improve complete response rate and to decrease relapse rate and large-scaled prospective studies to know natural history of long-term survivors.