The significance of carboxy-terminal telopeptide of type I collagen (ICTP) and osteocalcin (OC) in assessment of bone disease in patients with multiple myeloma.

Bone disease occurring in multiple myeloma is usually evaluated using radiological methods. These methods, however, provide not much information about the dynamic process of bone resorption and formation. This study analysed levels of serum markers of bone turnover (ICTP and OC), reflecting function of osteoclasts and osteoblasts. It demonstrates increased level of ICTP in 75 patients with MM compared to control group (8 persons) and patients with Waldenström's macroglobulinemia (10 persons). The level of ICTP was also higher in patients with more advanced bone disease and probably in higher stage of disease according to Salmon and Durie classification. This tendency was not observed in relation to OC. Result of the research confirms that ICTP may incur sensitive and specific markers of bone lesions in multiple myeloma.

[Hematological disorder caused by a giant myoma of the uterus]. / Zaburzenia hematologiczne wywolane miesniakiem macicy.

OBJECTIVES: Polycythaemia means an increase in the number of red blood cells and usually a corresponding increase in the haematocrit and in the quantity of haemoglobin. Regarding etiology, primary and secondary forms of polycythaemia can be distinguished. The primary form--polycythaemia vera--is caused by autonomous erythroblastic hyperplasia. Secondary polycythaemia--erythrocythosis--may arise as a consequence of erythropoietin (EP) overproduction due to tissue hypoxia. These changes may stimulate the EP synthesis in the kidneys. EP may also be produced in metaplastic cells. STUDY DESIGN: A 59-year-old woman was admitted to the Clinic of Haematology of the Wroclaw Medical University with a 10-years' history of significant increase in HB, VPRC and RBC. Physical examination showed an obvious plethora and a large intra-abdominal painless tumour. Laboratory data revealed increases in Hb, VPRC, RBC and EP. A bone marrow aspiration was normal. During surgery, a large leiomyoma of the uterus was found and a total hysterectomy was performed. Pathological examination showed the tumour to be fibroleiomyoma. 4 weeks after surgery Hb, VPRC, RBC and EP, all returned to normal levels. RESULTS: Excluding the effect of the renal compression, it can be concluded that erythropoietin secreted by the the tumour caused the secondary haematological changes.

[Diagnostic difficulties in Gaucher disease: report of two cases]. / Trudnosci w rozpoznawaniu choroby Gauchera: opis dwóch przypadków.

Diagnostic difficulties of Gaucher disease, a disorder resulted from a deficient activity of glucocerebrosidase is reported. Gaucher disease was described in the 16 year old male, 5 years after manifestation of the very first symptoms (fracture and osteomyelitis). At the age of 14, the cirrhosis due to viral hepatitis accompanied with splenomegaly was diagnosed. This findings was not associated with the earlier osseous disorders. Histopathologic examination of the removed spleen facilitate the diagnosis. The second case refers to 20 year old female. Clinical symptoms and additional test pointed to malignant neoplasm of thyroid, the reproductive organs or cancer of indistinguishable primary focus with metastases in the liver. Trepanobiopsy of bone marrow had made an accurate diagnosis possible, while determination of beta-glucosidase activity in peripheral white blood cells, chitotriosidase activity, and molecular investigations of gene specific to beta-glucocerebrosidase proved it.

Treatment-induced changes in 31P-MRS (magnetic resonance spectroscopy) spectra of sera from patients with acute leukemia.

31P-nuclear magnetic resonance (NMR) spectra were obtained in vitro from sera of 40 healthy volunteers and 30 patients with acute leukemia (AL) at the time of diagnosis and repeated up to 2-13 times during therapy. All spectra consisted of inorganic phosphate (Pi) peak (used as a reference peak) and two peaks from phospholipids (PL): one peak due to phosphatidylethanolamine and sphingomyelin (PE + SM) and second peak due to phosphatidylcholine (PC). Prior to initiation of therapy 31P spectra of sera of patients with acute leukemia differed from spectra of sera of normal individuals. Peak intensities of the PL were low in relation to Pi. During therapy leading to remission, resonance from PL progressively increased approximately to the spectral pattern in normal sera. Contrary to that, in non-responders the intensities of the phospholipids peaks remained unchanged. Long-term follow-up 31P-MRS studies showed not only a good correlation between this 31P-MRS evolution of sera and the response to the therapy but also showed changes in phospholipids' levels in the following days during and after therapy. Moreover, correlations were found between high-density lipoprotein (HDL), cholesterol (CHOL) and low-density lipoprotein (LDL) concentrations measured by conventional techniques and peak intensities of PC and of PE + SM acquired by 31P-MRS.

Application of 31P NMR spectroscopy in clinical analysis of changes of serum phospholipids in leukemia, lymphoma and some other non-haematological cancers.

The sodium salt of cholic acid added to serum caused separation of three phospholipid peaks located upfield from inorganic phosphate (Pi) due to phosphatidylethanolamine with sphingomyelin (PE + SM), lysophosphatidylcholine (LPC), and phosphatidylcholine (PC). 31P NMR spectra were obtained from the sera of 15 healthy volunteers, 10 individuals suffering from acute leukemia, 4 persons with malignant lymphomas, 13 patients with digestive tract tumors and 5 with renal cell carcinoma. The present studies confirmed our previous observation that the 31P spectra of sera of patients with acute leukemia and malignant lymphomas revealed a significant decrease in the phospholipid level at the time of diagnosis and displayed a good correlation between spectral parameters and stage of disease in patients responding and non-responding to therapy. Contrary to the preliminary studies, peaks from PE + SM and PC, and also a peak from LPC were observed. Changes in phospholipids in the 31P NMR spectra observed in patients suffering from digestive tract tumors and renal cell carcinoma were primarily dependent on the advance of the disease. Most of our patients with these cancers were in the early stage of the disease, and the spectra showed statistically significant decrease only in the LPC peak area, and no statistically significant changes of peak areas of PC and PE+SM in comparison to the control group. In conclusion, we can state that the LPC peak area is the most sensitive indicator in the monitoring of treatment in acute leukemia and malignant lymphomas. Our results also showed that LPC peak areas were decreased in the early stages of digestive tract tumors and a renal cell carcinoma.

Application of 31P NMR spectroscopy to monitor chemotherapy-associated changes of serum phospholipids in patients with malignant lymphomas.

31P spectra were obtained from 22 healthy volunteers and 35 patients with malignant lymphomas. Sera from patients were collected at the time of diagnosis and at several time-points during therapy. Long-term follow-up studies showed a good correlation between the 31P NMR spectra of sera and the clinically evident response of the disease to the chemotherapy. During therapy leading to remission resonance from phospholipids increased progressively resulting in spectra similar to those seen in normal sera. By contrast, in patients who did not respond to therapy the intensities of the phospholipid peaks remained relatively low or became progressively reduced as the disease progressed. To understand the source of the spectral differences, we also examined the concentrations of high-density lipoprotein, low-density lipoprotein, cholesterol, and triglycerides. In individuals responding to the treatment, both high-density lipoprotein and cholesterol increased to the point where they were statistically equivalent to those from healthy volunteers.

Chemotherapy-associated changes in 31P MRS spectra of sera from patients with multiple myeloma.

31P NMR spectra were obtained from sera of 22 healthy volunteers and 20 patients with multiple myeloma at the time of diagnosis and repeated up to five times during therapy. All spectra consisted of a Pi peak (used as a reference peak) and two peaks from phospholipids (PL): one peak due to phosphatidylethanolamine and sphingomyelin (PE + SM) and a second peak due to phosphatidylcholine (PC). Prior to therapy, peak intensities of the phospholipids were low relative to Pi. During therapy leading to remission, the resonance from PL progressively increased to approximate the spectral pattern seen in normal sera. By contrast, in non-responders an opposite trend was noted: the intensities of the phospholipid peaks became progressively reduced or remained unchanged. Long-term follow-up studies showed a good correlation between this 31P MRS evaluation of sera and the response of the disease to the therapy. In addition to the correlation with tumor response, our studies also show significant correlations between area, intensities of peaks of PE + SM, PC, and the concentrations of high-density lipoprotein (HDL) (correlation coefficients 0.46, 0.43, 0.59, respectively; p < 0.001). We found that the concentration of HDL in serum of patients with multiple myeloma was significantly reduced. In individuals responding to therapy HDL levels increased to the point where there were no statistically significant differences between them and healthy volunteers. In patients not responding to therapy, HDL concentration did not increase.(ABSTRACT TRUNCATED AT 250 WORDS)

[Long-term (over 10-years) survival in Hodgkin's disease]. / Dlugotrwale (ponad 10-letnie) przezycie w chorobie Hodgkina.

61 patients with Hodgkin's disease, i.e., 40.9% of all HD pts hospitalized in the years 1970-1981 are more than 10 years survivors: 46 are alive and 15 died of underlying disease or its complications. The most important prognostic factor at diagnosis was clinical advancement and the form A or B of HD. Age and sex also influenced survival but to a lesser degree. Patients living in CCR were more likely to have MC histology than those with relapsing disease, who more often showed LD and LP type. Among 5 persons with second neoplasms four disclosed NS type of HD. No statistical differences in clinical prognostic factors were encountered between further alive and those who died after more than 10-yrs. Almost all patients were able to normally continue their familial and professional lives.

[Lymph node involvement as a primary localization of extramedullary blast crisis of chronic granulocytic leukemia (PBS)]. / Zajecie wezlów chlonnych jako pierwotna, pozaszpikowa lokalizacja przelomu blastycznego przewleklej bialaczki szpikowej (PBS).

Among 28 patients with blastic crisis of chronic granulocytic leukemia (CGL) its primary symptom in four persons was lymph nodes enlargement. In two of them histopathologic examination confirmed myeloblastic infiltration. Lymph nodes involvement appeared 1-7 months before the onset of blastic transformation in peripheral blood and bone marrow: myeloblastic in two patients, promyelocytic in one and lymphoblastic myeloblastic (bi-phenotypic) in one. Survival time from nodal infiltration was 8-16 months and from the diagnosis of CGL 16-105 months. Remission of the disease after first blastic crisis was achieved in three patients. Cytogenetic examinations disclosed double Ph chromosome in three and single Ph chromosome in one patient.

Tracheo- and bronchoesophageal fistulas in Hodgkin's disease.

Among 330 patients with Hodgkin's disease, in five development of fistula between the respiratory and alimentary tract was observed; tracheoesophageal in three and bronchoesophageal in two patients. Generalization of the disease was the cause of fistula in three patients; it appeared as a result of necrosis of the neoplastic infiltration during cytostatic treatment or radiotherapy. In the fourth patient the fistula was of iatrogenic origin (radiation induced damage), and in the fifth it was the result of secondary neoplasm (bronchial anaplastic cancer).

[Activity of natural cytotoxic cells in non-Hodgkin's lymphoma]. / Aktywnosc naturalnych komórek cytotoksycznych w nieziarniczych chloniakach zlosliwych.

Cytotoxic activity of NK cells in the peripheral blood has been determined in 30 patients with malignant non-Hodgkin lymphomas prior to and following therapy. In the whole group as well as in subgroups classified according to the criteria of Working Formulation as lymphomas of the low, moderate and high degree of malignancy, activity of NK cells has been statistically significantly lower than that in healthy individuals. Marked increase in this activity has been noted in 19 patients in the state of clinical remission after the treatment with cytotoxic agents, and sometimes radiotherapy. The value of mean cytotoxic activity reached normal limits in the lymphoma of high degree of malignancy, and exceeded these limits in the lymphomas of moderate and low malignancy.

[Early results of the treatment of Hodgkin's disease in adults using the seven-drug cytostatic protocol]. / Wczesne wyniki leczenia choroby Hodgkina u doroslych wedlug siedmiolekowego protokólu cytostatycznego.

65 adult patients with Hodgkin's disease were treated acc. to multidrug protocol proposed by I. Koza et al. including doxorubicin, vincristine, vinblastine, bleomycin, procarbazine, lomustine and prednisone. 4 drugs (3 cytostatics and prednisone) including cycles were given repeatedly every 4 weeks. In the group of first line therapy (39 persons) 61.5% CR and 23% PR was obtained i.e. 84.5% therapeutic responses. The protocol used as salvage therapy resulted in 30% CR and 23% PR. Undesirable gastrointestinal effects were observed less commonly than after ABVD and CVPP schemes but myelosuppressive effect was more often seen and required attenuation of cytostatic drugs doses in the further cycles of therapy. The considered protocol seems to be a valuable one approaching to -but not achieving- the results of MOPP and ABVD schemes: is better tolerated because of elimination of strong emetic cytostatics (chlormethine and decarbazine) but late toxicity could be evaluable only after several years.

Haemolytic crises caused by Hoya carnosa in a patient with G6PD deficiency.

Haemolytic crises caused by contact with Hoya carnosa were observed in a 45-years old woman. The investigation of erythrocyte enzymes displayed both G6PD deficiency and its diminished affinity to G-6-P. Some properties of dehydrogenases isolated from erythrocytes of the patient's brother and son were also changed although clinical signs of haemolysis were absent.

[Disorders in the menstrual cycle and fertility of women with Hodgkin's disease]. / Zaburzenia miesiaczkowania i rozrodczosc kobiet chorych na chorobe hodgkina.

Sixty two women with Hodgkin's disease were questioned about disorders in their menstrual cycle and fertility. Menstrual cycle disorders were noted by 38 respondents, i.e. over 60%. Amenorrhoea persisted in 24 of these women. These disorders were significantly more frequent in patient over 30 year of age. There were 52 labours in the group of 38 women with menstrual cycle disorders prior to them, and 21 labours in a group of 24 women without any abnormalities. Hodgkin's disease was diagnosed in 6 patients during pregnancy: 2 of them were treated with irradiations on the upper part of the body while 4 remained untreated. After radiotherapy, there were 2 deliveries in women of the group with menstrual cycle disorders, and 9 deliveries in women without such disturbances. Answers to the supplied questionnaire did not provide any information on the congenital abnormalities in neonates.

[Effect of livex on hypochromic anemia]. / Wplyw livexu na niedokrwistosc z niedoboru zelaza.

Livex is a lyophilised preparation of the bovine blood which contains 70% of proteins and 0.3% of iron. Livex was given to 25 patients with iron deficiency anemia (group I), and 25 patients with lymphoreticular malignancies accompanied by iron deficiency with or without anemia. The dose was 5 g t.i.d. for 3 months. The majority of patients of group I were women. Excessive menstrual bleeding and chronic gastritis were most common causes of iron deficiency in this group. Livex statistically significantly increased hemoglobin, hematocrit, corrected reticulocyte count, mean erythrocyte hemoglobin, serum iron, percent transferrin saturation, and leukocyte count. Therapy produced significant increase in serum iron, total iron binding capacity, and percent transferrin saturation in patients with lymphoreticular malignancies.

[Hodgkin's disease and non-Hodgkin's lymphoma in 2 sisters]. / Ziarnica zlosliwa i chloniak nieziarniczy u dwóch sióstr.

For the first time in the Polish medical literature an appearance is presented of malignant lymphogranulomatosis and of a non-Hodgkin lymphoma in two sisters. A discussion on the etiology of the condition is presented on the ground of the cytogenic studies and tissue compatibilities antigens as well as of the literature of the subject. Genetic background of the two different diseases in two sisters is stressed.

Prognostic significance of secondary cytogenetic changes and nonspecific cross-reacting antigen (NCA) in patients with Ph-positive chronic myeloid leukemia.

Cytogenetic analyses were carried out on peripheral blood and bone marrow cells of 31 chronic myeloid leukemia (CML) patients who presented with blastic, accelerated, or chronic phases. The percentage of cytoplasmic nonspecific cross-reacting antigen (cNCA, a marker of myelocytic differentiation)-containing cells was determined in the same blood or bone marrow samples. The patients were divided in two groups according to cytogenetic results: those with aberrations in addition to the Philadelphia chromosome (Ph1) and those with Ph1 only. Among the additional aberrations such changes, not typical of CML, were found: del(2)(p21), t(6;11)(q25;q23), and t(12;?)(p13;?). The survival time and the percentage of cNCA-positive cells of patients in blastic and accelerated phases were compared between the above-mentioned two groups of patients using the Student t test and the Kaplan-Meier estimator. The percentage of cNCA-positive cells was significantly lower and the survival time significantly shorter in the group of patients with additional aberrations. The probability of survival according to the Kaplan-Meier estimator was also lower for this group. These data suggest that the immunologically determined lower degree of maturity, that characterized cells bearing additional aberrations, coincides with and/or results in more rapid progression of the disease.