Chronic neutrophilic leukemia(CNL) is a very rare myeloproliferative disease, characterized by sustained mature neutrophilic leukocytosis with granulocytic bone marrow infiltration, high NAP(neutrophilic alkaline phosphatase) score and absence of philadelphia chromosome, It is frequently accompanied by hepatosplenomegaly, elevated serum vitamin B_12 and uric acid level. For the diagnosis of CNL, the leukemoid reaction, especially secondary to neoplasia, infection and autoimmune diseases, should be excluded.

Since Tuohy's first description in 1920, more than 50 cases fullfilling the above criteria have been reported worldwide, and 4 cases in Korea. Several authors have demonstrated the defect of intracellualr killing in the mature neutrophil and this finding correlates well with the very high incidence of fatal infection, The hemorrhagic diasthesis in CNL is caused by functional abnormality of the platelet. This disease has tendency to transform to blastic crisis and acute leukemia as in other myeloproliferative disease, but characteristically shows frequent coexistence with multiple myeloma. Until now , the therapeutic trials in CNL have been disappointing. Hydroxyrea and busulfan can control hyperleukocytosis. On the basis of functional defect in neutrophil, alpha-2b-interferon has been tried and several reports have demonstrated the clinical and functional effect of interferon on CNL.

CNL is very rate hematologic disease and there are few report about general aspect of disorder. We report here a typical CNL case presenting with splenomegaly and leukocytosis with a review of the literature.

Choriocarcinonla is very rare malignancy, accounting for less than 1% of all testicular germcell tumor. However, it is an important disease in the field of oncology, as it represents ahighly curable malignancy. and one in which the incidence is focused on young patients attheir peak of productivity. In nonserninomatous germ cell testis tumor, assessment of prognosticfactors is related to develop a basis for more rational therapy for each individual patient.Along with prognostic staging, appropriate treatment shoud be applied to each patient to improve disease-free survival. And. surgical resection of residual masses after cisplatin-based chemotherapy is an established adjuvant to chemotherapy, because complete remission can be improvedabout 10% with appropriately timed complete resection of residual diseases. So, we reporta case of a 27-year old male patient with testicular choriocarcinoma who presented with multiplelung metastases after radical orchiectomy. He recieved lung wedge resection after 8 cycles ofcisplatin, etoposide, ifosfamide combination chemotherapy. and complete remission was confirmed and maintained.

Citations

Citations to this article as recorded by  

• Impact of Lactate Dehydrogenase and Hemoglobin Levels on Clinical Outcomes in Patients With Paroxysmal Nocturnal Hemoglobinuria: Results From the National Korean PNH Registry
  Jun Ho Jang, Jin Seok Kim, Cindy Thiow Koon Lim, Nora J. Kleinman, Karl-Johan Myren, Alice Wang, Yogesh Patel, Jong Wook Lee
  Journal of Korean Medical Science.2024;[Epub]     CrossRef

Although the role of surgical management of metastatic disease from primary carcinoma of the coln and recutm is still controversial, resection of hepatic metastasis improves survival rate of patients with primary colorectal carcinoma treated locally. The lung is the most common site of extra-abdominal metastasis following resection of a prymary colorectal tumor and not amenable to curative therapy.

It is possible to resect the pulmonary metastasis in selected patients following resection of colorectal cancers, but the 5-year survival rates are ranged from 9% to 57%. Authors report a case of resection of pulmonary metastasis occured 3 years after resection of primary colon carcinoma.

Among the hematologic complications of solid tumors, leukoerythroblastosis, DIO and MAHA have been known to be clinically significant According to the recent increasing incidence of cancer, these hematologic complications have been reported frequently. However, the simutaneous developement of such manifestations is rare. Recently, we have experienced a patient with gastric adenocarcinoma in which case severe bleeding tendency were observed and laboratory studies including bone marrow examination demonstrated the findings of leukoerythroblastosis, DIC and MAHA. She died of upper gastrointestinal bleeding despite the supportive care such as vlood transfusions. We should give attention to the findings of peripheral golld smear for the patient with malignancy and it is mandatory for therapeutic plan to perform bone marrow examination if the above mentioned hemorrhagic complications are suspected.

Selective absence of serum IgA is the most commonly diagnosed form of human immnndeficiency disease, being reported in about one in 500-700 subjects in population surverys. Datailed clinical and laborattory studies of subjects with selective IgA deficiency generally reveal significantly increased incidence of sinopulmonary infection, gastrointestinal symptomatology, autoimmune disease and autoantibodies.

Authors experienced selective IgA deficiency associated with an scleroderma in a 25 years old young woman. She had pulmonary tuberculosis, high IgE level, reactive rheumatoid factor, positive for antinuclear antibody, and no presence of anti-IgA antibody.

Reliable venous access is increasing serious problem in cancer chemotherapy Patients.

Thiry six implantable second generation catheter system(A. Port) were placed subcutaneously in 35 patients with cancer.

A. Port were in place for an average of 158 days(range 10-731⁺ days). Complications included infections in 3 cases, thrombosis in 1 case, skin laceration in 1 case and pneurnothorax in 1 case. Five ports were removed due to complication. One pneumothorax was managed conservatively;

It is concluded that A. Port implantation constitute a safe and convenient access with a low rate of complication for long term intravenous chemotherapy in cancer patients.

To evaluste the efficacy and toxicity of IEP chemotherapy in locally advanced and metastatic non-small cell lung cancer, nineteen previously untreated patients with non-small cell lung cancer were studied.

Patients received ifosfamide 1000mg/m² with mesna, 20% of ifosfamide dose at 0,4, 8 hours after ifosfamide, etoposide 100mg/m², and cisplatin 20mg/m² with full hydration and mannitol diuresis intravenously on days 1-3 every 4 weeks.

Eighteen male and one female, two stage IIIA, 10 stage IIIB and 7 stage IV patients have been treated.

The response rate of IEP was 26% with no complete remission, 53% of stable disease, and 23% of progressive diasease.

The overall median survival was not reaced yet and 20 month survival rate was 51.4%, The duration of remission was at the range of 4⁺ to 40 weeks.

The toxicities were alopecia(100%), nausea and vomiting(47%), leukopenia(14%), infection(6%), renal dysfunction(6%) and oral mucositis(5%).

This phase II study of IEP in advanced non-small cell lung cancer is not superior to another combination regimens in terms of response but toxicity was tolerable. Other new chemotherapeutic drugs and newer trials should be developed further.

In the past gastric carcinomas composed predominantly of signet ring cells formed only a small percentage of gastric malignancies. However. we have had the impression that in recent years the relative incidence and amount of signet ring cells in biopsy and resection specimens of gastric carcinoma have risen sharply. This report is an analysis of 69 cases of signet ring cell type of gastric adenocarcinoma diagnosed at Ewha Womens University Hospital from January, 1987 to December, 1991. The results obtained were summerized as follows.

1) The male to female ratio was 1:1.2.

2) The peak age incidence was the 6th decade and the 7th decade with 23.9% each other, and the 8th decade with 17.4%, the 5th decade and the 4th decade with 16% each other. Average age of patients was younger than entire gastric adenocarcinoma.

3) The major clinical symptom was epigastric pain, indigestion, vomiting and abdominal fullness in decreasing orders.

4) The macroscopic feature of the signet ring cell type of adenocarcinoma by gastronberscopy showed Borrmann's type III 26 cases(37.6%), type IV 21 cases(30.4%), type II 10 cases(14.5%).

5) The location of signet ring cell type was mainly the gastric antrum 39 cases(56.5%), the body 18 cases(26%), and widespread from body to antrum 9 cases(13%).

6) The size of lesion showed above 3cm with 53%, 1.5~3.0cm with 31.3%, below 1.5cm with 15.7% in frequency.

7) Thirty out of 39 cases(76.9%) involve to the serosa, 3 cases(7.7%) to the muscle layer, 3 cases(7.7%) to the mucosa and the submucosa each other.

8) Distant metastases were relatively frequently occurred in increased CEA group.

9) Five-year survival rate of 49 cases was 62.8%.

With development of new technology such as handing technique and high resolution banding technique, cytogenetic examination has been utilized greatly in diagnosis and treatment of patients with hemato-oncologic diseases.

Forty two patients with newly diagnosed and previously untreated hemato-oncologic diseases were examined for chromosomal abnormalities which included 11 cases of acute myelogenous leukemia(AML). 7 cases of acute lymphoblastic leukemia (ALL) and myelodyspalstic syndrome (MDS) each, 6 cases of chronic myelogenous leukemia (CML). 5 cases of other myelolproliferative disorders(MPD). 4 cases of non-Hodgkin's lymphoma(MHL). and 1 case each of aplastic anemia(AA) and malignant schwannoma(MS).

Two cases of AML(18.2%) showed 45 XO and aneuploidy. Four cases of ALL(55.5%) showed chromosomal abnormalities : 3 cases with Philadelphia chromosome and 1 case with 45 XO.

Only one case of MDS(14.3%) showed trisomy 8 and none of 3 cases which were converted to AML showed chromosomal abnormalities. Five cases of CML(83.3%) showed Philadelphia chromosome. Chromosomal abnormalities were not noted in cases of MPD, MHL, AA and MS.

Collective studies of chromosomal abnormalities in hemato-onclologic diseases should be investigated further.

Priapism is the pathologic prolongation of a penile erection most often associated with pain but not with sexual excitement or desire. Recently we experienced one case of priapism accompained by chromic myelocytic leukemia.

For treatment of priapism, cavernososponsiosal shunt, radiotherapy and chemotherapy with hydroxyurea were done.

Since the national insurance system has implemented in 1977. the aspect of supply and demand in terms of treatment has been solved to a certain degree. but the health of the people has not been satisfied. Furthermore. considering the efficiency of cost-effective analyais. the development in the health promotion of medical field has gradually come to be called for.

As a part of the research, we have established the the major adult disease of our country. such as hypertension, diabetes, gastric cancer and cervical cancer, elicited the items of knowledge. attitude and practice(KAP) aimed at the prevention and early diagnosis of the above disease, prepared self-questionires. and made the persons concerned evaluate the KAP for their health.

We think that the utilization of the KAP questionires is effective for the promotion of health by the self-efficacy consciousness. and that the KAP itself is greatly significant as an excellent materials for public health education.

The sustained research and study which is to be followed is to go over the validity, reliability, efficacy. which would, make a great contribution to public health in the community.

Malignant schwannoma is a malignant neoplasm of nerve sheath cells. It develops in a solitary fashion or associated with Von Recklinghausen disease.

We experienced a malignant schwannoma in a 20-year old man with Von Recklinghausen disease. The lesion was gure, irregular, cystic and solid mass with septations in the right retroperitoneal region, inferior to the kidney. The histologic findings of retroperitoneal and pulmonary mass was compatible with malignant schwannoma. Incomplete excision due to infiltration to the adjacent tissue, radiotherapy and combined chemotherapy were performed.

We report a case of retroperitoneal malignant schwannoma associated with Von recklinghausen disease.

Twenty nine patients with small cell lung cancer patients has been studied from January 1986 to September 1990. In 14 limited disease patients, combination chemotherapy combined with radiotherapy to primary lung, mediastinum, supraclavicular lymph nodes and prophylatic whole brain irradiation was performed.

Fifteen extensive disease patients were treated combination chemotherapy alone.

The results obtained were as follows :

1) Coughing, dyspnea and chest pain were frequent symptomes in the order of frequency. Syndrome of inappropriate antidiuretic hormone secretion and Eaton-Lambert syndrome were observed in 20.7% and 19.0% respectively.

2) A 92.8% overall response rate and 50.0% overall complete remission rate in limited disease and 53.0% overall reponse rate width 40.0% complete remission rate in limited disease and 53.0% overall reponse rate with 40.0% complete remission rate in extensive disease were achieved. Overall response rate of limited disease was higher than that of extensive disease.

3) Overall median survival was 10.9 months and 11.1 months in limited diease and 8.4 months in extensive diease were obtained. Overall median survival of complete responder was 11.7 months, that was longer than 6.0 months of partial responders.

4) The treatment toxicities were mainly nausea, vomiting and alopecia but fatal one episode or arganulocytosid was experienced. Further trials and new therapeutic plans are must be set to improve therapeutic efficary and minimize toxicities.

Stomach cancer is the most common malignant neoplasm in Korea. and 5-year survival rate of early cancer is execellent in comparison with advanced cancer. Therefore, tremendous effrots should be made for the early detection of gastric cancer.

The patients had presenting sysmptoms indistinguishable from those of benign peptic ulcer disease, so radiology or endoscopic examination with multiple biopsies should be performed.

The author reviewed 22 cases collected from Ewha Woman University Hospital, from Jan. 1983 to Apr. 1990.

The results were sumerized as follows ;

1) The incidence of EGC among gastric cancer was 5.1%.

2) The over-all male to female sex ratio was 1 : 1.6, and the mean age was 50.2% years old.

3) The most common symptom was epigastric pain(77.8%) and the most common duration of symptoms was below 3 months(41%).

4) The diagnostic accuracy was 68.2% endoscopically and 70.3% radiologically.

5) The most frequent morphologic type of EGC was II_c(45.5%).

6) The major location EGC were pylorus and antrum(59.1) and and lesser curvature(50.05).

7) The cancer was confined to regional lymph node was found in 13.6%.

8) Thd mose common microscopic tissue type of EGC was moderately differentiated adenocarcinoma(36.4%).

9) Of 22 cases, all who had received gastric resection, were survived.

It has been recognized for a long time that human malignant diseases are associated with immune deficiency states. Impaired phagocytic function, low levels of plasma opsonins, impaired bactericidal activity of granulocytes and macrophages, deficiency of myeloperoxydase, reduced migration of polymorphonuclear leukocytes and granulocytopenia have all been found in malignant condition primarily or secondarily.

Especially neutrophils are crucial to the body's defenses against infection. Defects in phagocytes can contribute to the local establishment and systemic spread of serious bacterial infection.

The authors studied neutrophil function in 40 control and 15 patients with acute myelogenous leukemia, 10 with non-Hodgkin's lymphoma, 40 with stomach cancer, 10 with hepatoma, 10 with pancreas cancer and 25 with other organ tumor to prove the mechanism of increased risk and the severity of infection using a battery of simple and reproducible neutrophil function tests as adherence to nylon wool fiber, migration under agarose method and nitrobule tetrazolium reduction test.

The results obtained are as follows;

1) On adherence to nylon wool fiber, the adherence was significantly decreased in patients with acute myelogenous leukemia, but increased in patients with lymphoma. In patients with other solid tumors, there were no significant differences from those of controls. After chemotherapy with Cisplatin and 5-FU, the adherence was significantly decreased than that of prior to therapy.

2) On migration under agarose, the chemotactic differential was significantly decreased in patients with acute myelogenous leukemia but increased in patients with hepatoma and pancreas cancer. Presence of factors of those increment was suspected. After infusion of Cisplatin, chemotactic differential was decreased significantly but after infusion of 5-FU, it was decreased but there was no significance.

3) On NBT reduction test, NBT reduction was diminished in patients with acute myelogenous leukemia but increased in patients with hepatoma and pancreas cancer. It is assumed that some unidentified factors would be present to enhance chemotaxis and NBT reduction in patients with hepatoma and pancreas cancer. After infusion of Cisplatin, NBT reduction was significantly decreased, but after infusion of 5-FU it was decreased but not significantly.

In patients with acute myelogenous leukemia, neutrophil functions were much impaired but in patients with solid tumors, there were no neutrophil function defects.

After chemotheraphy with Cisplatin and 5-FU, neutrophil functions were much compromised and this would predispose to increased risk of infection.

Gilbert's Syndrome is the name most frequently used to describe a condition which has been called constitutional heptic dysfunction, familial nonhemolytic jaundice of icterus intermittens juvenilis. It is characterized by a benign, persistent, but variable elevation of the plasma unconjugated bilirubin. A 27-year-old man was admitted because of general malaise, anorexia, sore throat and slight icteric sclera. On physical examination, there were mild icteric sclera and slightly injected pharynx. The liver was palpable 1 finger breath BRCM and the spleen was also palpable I finger breadth BLCM. Hematologic studies revealed slight anemia with slightly elevated corrected reticulocyte count. Liver function tests were normal except for slightly elevated serum total bilirbin. Histolog-ic findings of the liver showed nonspecific findings of mild cholestasis. Reduction in caloric intake to 300 calrory a day for 72 hours resulted in a significant increase in the plasma bilirubin concentration(especially unconjugated form) in this patient. Now he has no subjective symptoms and leads a normal activity without specific treatment.

Citations

Citations to this article as recorded by  

• A Case of Congenital Hemolytic Anemia of Unknown Cause Combined with Gilbert's Syndrome
  Ji Whan Lim, Joon Hyouk Choi, Yang Hoon Nam, In Seok Seo, Seong Min Yoon, Myoung Sook Koo
  The Korean Journal of Hematology.2008; 43(1): 58.     CrossRef

A case of hypokalemic familial periodic paralysis is presented and the litures are reviewed. A 15-year-old boy was admitted to Ewha Womans University Hospital with chief complaints of paralysis of both extremities and trunk on awaking in the morning. He had a history of quadriplegia, 8 months ago. Laboratory study revealed a hypokalemia(1.6mEq/L) and biopsy specimen taken from the gastrocnemius muscle showed mild vacuolization. After treatment with potassium chloride, paralysis was completely recovered.

The authors studied 50 controls(25 male and 25 female) and 79 diabetes mellitus patients(38 male and 41 female) who were admitted to Ewha Womans University Hospital from June, 1983 to May, 1984 to investigate the disorders of lipid metabolism, hemoglobin A_1c and their correlaton. The results were as follows: 1) Age incidence was highest in the 6th decades. 2) Mean fasting blood sugar was 191.9±82.29mg% and postprandial 2hour blood sugar was 278.81±118.19mg% in male diabetes mellitus patients, and 196.48±68.94mg% and 261.16±85.84mg% in female diabetes mellitus patients. After control diabetes mellitus blood sugar levels were decreased significantly. 3) Hemoglobin A_1clevels at fasting and postprandial 2 hour were significantly higher than those of controls. And Hemoglobin A_1c level were progressivly decreased after 3 months' controlling of diabetes mellitus. 4) Pre-β and β-lipoprotein, triglycerides and cholesterol were significantly higher than those of controls but α-lipoprotein was lower than that of controls. After 6 months of diabetic control, pre β-lipoprotein, triglycerides and cholesterol were decreased. 5) Between Hemoglobin A_1c and fasting blodo sugar had positive correlation, an cholesterol and hemoglobin A_1c also had positive correlation, but those were not statistically significant.

Lipoprotein cholesterol and triglyceride levels have been determined innormal and diabetes. The abnormalities of lipoprotein were investigated by SDS-polyacrylamide gel electrophoresis in the serum and the 3 major lipoprotein classes for diabetes. HDL cholesterol levels were lower in the diabetes compared to the normals. VLDL triglyceride levels also elevated in the diabetes. There were significant shifts in the distribution of lipoprotein cholesterol, with an increase in LDL cholesterol and decrease in HDL cholesterol. The results of this study suggest that diabetes may be associated with changes in both lipoprotein triglyceride and cholesterol levels. The electrohoretic patterns of VLDL and HDL in diabetes showed the abnormal pattern in comparison to normal.

The maximum exercise testing using treadmill walking was performed in 36 patients of diabetes mellitus who were admitted to the Ewha Womans University Hospital from March, 1983 to August, 1983 and 30 normal control subjects to evaluate the efficacy of significance to detect latent coronary artery disease in diabetes mellitus as a chronic complication.

Among 36 patients of diabetes mellitus, ten(27.8%) were treadmill exercise test positive, of whom two had ST depression in resting electrocardiogram but eight didn't have any changes in resting electrocardiogram.

Since treadmill exercise test can be a useful sensitive diagnostic tool for early detection of latent coronary artery disease in the patients of diabetes mellitus as a chronic complication.

The authors experienced a 29 year old female patient with hypoplastic anemia during her illness of typhoid fever. Because that is very rare and unreported, we are reporting this patient. She was promptly recovered from hypoplastic anemia with antibiotics for typhoid fever.

No abstract available.

To evaluate the long term results of sequential neoadjuvant chemotherapy followed by radiotherapy in locally advanced non small ciell lung cancer(NSCLC), 32 patients with stage III NSCLC were studied.

Neoadjuvanjt chemotherapy was performed with 2-3 cycles of cisplatin containing combination at the interval of 3-4 weeks, and then radiotherapy to and primary tumor, mediastinum and both supaclavicular lymph nodes was followed.

The results were as follows:

1) After neoadjuvant chemotherapy, partial remission(PR) rate was 59.4% without complete remission(CR). At completion of radiotherapy, CR rate was 28.1%, PR rate 50.0% and overall response rate was 78.1%.

2) The median survival of all patients was 14.4 months, for stage III A NSCLC patients was 22 months compared to 11.4 month in stage III B NSCLC patients without significance. For responder of neoadjuvant chemotherapy, median survival was 16.6 months, and 9.3 months for the non responder(p<0,05). Median survival of responders at completion of chemotherapy was 26.8 months at the rangd of 4-48 months. Overall survival rate at 2 year was 27.1% and continued plateau till 50 months without late relapse.

3) The acute toxicities were tolerable in all patients and not fatal, but late complications of three restricitive pneumonitis and one cough induced syncopal attack compromise the quality of life among the 8 long term survivors who lived over 2 years.

4) The relapse rate was 58.1%(18 out of 31 cases) and the pattern of relapse was local in 14 cases(45.2%), systemic in 3 cases(9.7%) and local plus systemic in 1 case(3.2%). The sites of systemic relapse were brain in 3 cases and brain combined with bone in 1 case.

In conclusion, sequential neoadjuvant chemotherapy and radiotherapy can prolong the survival and diminish the systemic relapse in locally advanced non small cell lung cancer patients but local failure was the main problem in these patients.

To enhance the local control, other trials such as concurrent chemoradiotherapy, hyperfractionated radiotherapy combined with developing more effective new chemotherapeutic agents and combined regimens should be further investigated.