Elective splenectomy in relapsing thrombotic thrombocytopenic purpura.

Between 20 and 40% of surviving patients with thrombotic thrombocytopenic purpura (TTP) have relapses. Plasma exchange therapy is usually effective in treating relapses, but this treatment does not prevent TTP recurrence. The role of splenectomy in relapsing TTP is still controversial. We describe a patient with multiple relapses of TTP who was successfully treated with elective splenectomy.

Haemoperitoneum caused by haemorrhagic corpus luteum in a patient with type 3 von Willebrand's disease.

In female patients affected by congenital coagulation disorders haemorrhagic corpus luteum must be considered for the differential diagnosis of acute abdomen. We report the complication in a 22-year-old woman with type 3 von Willebrand's disease who presented with abdominal pain and haemorrhagic shock. Along with adequate factor VIII replacement therapy oral contraceptive treatment is the key for the management of this potentially life-threatening complication and the long-term prevention of future bleeding episodes of this origin.

Dapsone for refractory chronic idiopathic thrombocytopenic purpura.

Fifteen patients with refractory chronic idiopathic thrombocytopenic purpura (ITP) were treated with dapsone (100 mg/d) for 1-31 months. The overall response rate to dapsone was 40%. Five patients responded in 1 month and one patient in 2 months. No pretreatment characteristics--sex, age, platelet count or duration of ITP--were correlated with response to dapsone. Treatment was well tolerated. The most frequent adverse effect was dose-related haemolytic anaemia. In our experience, dapsone provides an inexpensive and well-tolerated alternative for patients with ITP who had inadequate responses to conventional therapy.

[Cutaneous B-cell lymphoma with aggressive clinical presentation: apropos of a case]. / Linfoma cutáneo de células B con presentación clínica agresiva: a propósito de un caso.

A patient with an atypical clinical presentation of cutaneous B-cell lymphoma is reported. He showed a rapidly growing tumour with areas of skin necrosis, haemorrhage, ulceration and suppuration in his upper back. There were involvement of contiguous anatomic site and extracutaneous spread. The histology showed diffuse lymphoid infiltration of the dermis. The cell morphology was that of a monotonous population of centroblasts. Treatment with CHOP and radiotherapy provided long-term complete remission. This case illustrates that primary cutaneous B-cell lymphoma may behave as a high-grade malignancy. Vigorous treatment with chemotherapy and radiotherapy is recommended in such cases.

Simultaneous determination of hemoglobin derivatives, oxygen content, oxygen capacity, and oxygen saturation in 10 microliters of whole blood.

We describe a new method for simultaneous determination of four hemoglobin derivatives (deoxyhemoglobin, Hb; oxyhemoglobin, HbO2; methemoglobin, Hb+; and carbon monoxide hemoglobin, HbCO) and total oxygen content in 10 microliters of whole blood. Percentage HbO2, HbCO, Hb+, total hemoglobin (Hbt), and oxygen capacity can also be obtained from the experimental data by simple calculations. Total analysis time is 1 min. Blood is diluted 100-fold with a buffer contained in a quasi-anaerobic cuvette, where simultaneous measurements of oxygen pressure (by a po2 electrode) and adsorbance (at 497, 565, and 620 nm) are made. The decrease of oxygen pressure, as recorded by the oxygen electrode, is proportional to the amount of deoxyhemoglobin. The concentrations of HbO2, HbCO, and Hb+ can be obtained from absorbance measurements at the specified wavelengths. The new method eliminates the use of short-path optical cells and, due to the low sample volume requirement, makes possible the automated measurement of hemoglobin derivatives and oxygen saturation in arterialized capillary blood.

[Analysis of 4 prognostic scoring systems in 197 myelodysplastic syndrome patients]. / Análisis de cuatro sistemas de puntuación pronóstica en 197 síndromes mielodisplásicos.

PURPOSE: To evaluate the prognostic significance of four scoring systems applied with predictive trends to myelodysplastic syndromes (MDS). MATERIAL AND METHODS: This study is comprised of 197 patients with MDS diagnosed in accordance with the FAB criteria and followed-up in our Department between Jan '75 and Dec '89. The following MDS subtypes were found: refractory anaemia (RA), 58 cases; sideroblastic refractory anaemia (SRA), 42 cases; refractory anaemia with excess of blasts (RAEB), 46 cases; RAEB in transformation (RAEB-T), 39 cases; and chronic myelomonocytic leukaemia (CMML), 12 cases. The following scoring systems were applied: Mufti's 1985, Varela's 1985, Sanz's 1989 and our own of 1991. The statistical analysis was performed according to Kaplan-Meier actuarial system and the log-Rank test of actuarial survival. RESULTS: (1) Three groups (A, B and C) can be defined by the Bournemouth system, with median survivals of 57.6, 17 and 7.6 months, respectively. The majority of cases (118) were included in group B. Group A has not reached 25% of actuarial survival probability, whereas groups B and C did at 32 and 10 months, respectively. With regard to the morphologic subtypes, RA and SRA were included in groups A and B, and RAEB, RAEB-T and CMML pertained mostly of group C. Sixty cases (90.9%) evolving into acute leukaemia (AL) corresponded to those last groups. (2) The three groups defined by Varela's system (0-1, 2-5 and 6 or more) have median survival of 85.6, 24 and 14 months, respectively. Like in the former system, group 0-1 has not reached 25% actuarial probability, this appearing at 70 and 20 months, respectively, in groups 2-5 and greater than 6. The distribution of the cytological varieties, RA and SRA amongst the groups is heterogeneous although there are more common within the cases included in groups 0-1. All the cases evolving into AL were included in the groups 2-5 and greater than 6. (3) The 3 groups of the system proposed by Sanz (0-1, 2-3 and 4-5) had median survival of, respectively, 58, 15 and 14 months. Like in the preceding cases, group 0-1 has not reached the 25% actuarial probability, while this figure appears at 28 months for group 2-3 and at 20 months for group 4-5. RA and SRA varieties are included chiefly in group 0-1, while RAEB and RAEB-T appear mostly in groups 2-3 and 4-5. The distribution of the cases and evolving into AL in heterogeneous according to this system, although they predominate in groups 2-3 and 4-5. (4) The prognostic groups are defined by the system proposed by us (namely 0-2, 3-5, 5 greater than or equal to 6) with median survivals of 89.3, 17 and greater than 11 months, respectively. Striking difference was seen when studying the cumulated survivals observed, on each of the three percentages considered, between the groups. The different cytological varieties distributed reasonably with higher incidence of RA and SRA in group I and RAEB, RAEB-T and CMML in group III. This system offers statistical significance when comparing RA with SRA, RAEB with RAEB-T and, obviously. RA+SRA with RAEB+RAEB-T+CMML. The evolution into AL showed also statistical significance with respect to the three groups. CONCLUSIONS: 1. Three prognostic groups regarding the patient's survival could be established in our series with all the scoring systems analyzed, except that of Sanz. 2. Low risk (RA and SRA) and high risk (RAEB, RAEB-T and CMML) cytological varieties can be identified with all the scoring systems; that of Sanz is also capable of discriminating RA from SRA. 3. Some prediction on the possibility of evolution into AL can be attained of with each system. 4. The scoring system proposed by us shows higher discriminating capability on the cytologic varieties as well as higher predictive value on the possibility of evolution into AL. Nevertheless, it must be evaluated in other series in order to reach general acceptance.

[Non-Hodgkin's lymphomas. II. Analysis of the prognostic factors in a series of 307 patients]. / Linfomas no hodgkinianos. II. Análisis de los factores pronósticos en una serie de 307 enfermos.

PURPOSE: To analyse different clinico-biologic data in order to assess their prognostic value in non-Hodgkin's lymphoma (NHL) patients. MATERIAL AND METHODS: The series comprises 307 patients with NHL diagnosed and treated between 1975 and 1988. The histopathologic diagnosis was revised in accordance with the working formulation system, three prognostic groups being thus considered: low-grade (LGL), intermediate-grade (IGL) and high-grade (HGL) lymphomas. Age, sex, clinical course prior to diagnosis, presence of B symptoms, histologic type, number of lymph-node areas involved, bulky disease, anaemia, thrombocytopenia, LDH, stage and response to therapy were all evaluated for the study. Survival curves were drawn with the Kaplan-Meier method, and the log-rank test was used for comparison of median survival. Whenever the univariate analysis achieved statistical significance, a multivariate analysis was performed by means of a multiple correlation and regression study in accordance with the Cox's model, in which the variables were expressed in a binary model. RESULTS: The following 8 values were found significant in the univariate study of low-grade lymphomas: age, number of involved areas, bulky disease anaemia, thrombocytopenia, LDH, stage, and initial response to treatment. In intermediate-grade lymphomas, the significant findings were age, number of affected areas, bulky disease, thrombocytopenia, LDH, stage, and initial response. For high-grade lymphomas, number of affected areas, thrombocytopenia, LDH, stage and initial response were found statistically significant. Although no significant differences were found for survival within each of the three grades, such differences were significant between them. In the multivariate analysis, age was significant only in the LGL (p < 0.0001) in IGL, age (p < 0.07) and initial response to therapy (p < 0.0001) achieved significant value, and in HGL, stage (p < 0.02) and initial response to treatment (p < 0.0001) attained significance. CONCLUSIONS: The univariate analysis provides various prognostic factors of statistically significance, as reported in the literature, but these after the multivariate analysis was applied, were reduced to age, stage and initial response to treatment.

Chronic idiopathic thrombocytopenic purpura in the elderly.

From a group of 118 patients with chronic idiopathic thrombocytopenic purpura (ITP), 43 were older than 60 years at diagnosis. In this report, we describe the clinical evolution and therapeutic response in young and old patients. The overal rate of hemorrhagic manifestations was similar in the two age groups, but a greater risk for severe bleeding was observed in elderly patients. There were no significant differences between old and young patients in response to steroids. In none of our patients was mortality associated with bleeding or side effects of the treatment. In conclusion, we have observed a more benign clinical course in elderly patients with chronic ITP as compared to previous reports.

[Monoclonal gammopathies of undetermined significance. Clinical course and biological aspects of 397 cases]. / Gammapatías monoclonales de significado indeterminado. Aspectos clínicos, biológicos y evolutivos de 397 casos.

The diagnostic criteria, incidence, clinical characteristics and outcome of 397 patients with monoclonal gammopathies of undetermined significance, all of them diagnosed and followed-up at the Haematology Service of the Miguel Servet Hospital, in Zaragoza, between January 1970 and December 1988, were revised. The patients' mean age was 64.7 years (range: 2-89). The M/F ratio was 236/161. The mean concentration of the M component (MC) was 1.17 g/dL (range: 0.20-3.50), this being under 0.50 in 65 cases. IgG was the most frequent MC (71.26%), followed by IgA (14.34%) and IgM (10.82%). Multiple MC was present in 14 cases (3.58). Light chains were passed in urine by 33 patients (8.31%). No associated pathology was found in 213 patients (53.65%) upon MC discovery, while 65 other (16.31%) were carriers of different blood disorders, chronic lymphoproliferative diseases being the commonest (11.57%). In 30 patients (7.30%) the MC was associated to nonhaematological malignancies, and 29 others had an underlying chronic infection. Chronic liver disease was present in 25 cases, and autoimmune disease in 14. Transient monoclonal gammopathy was seen in a small group of patients (6.54%), most of them suffering from acute infectious illness. With regard to the group of patients without any associated pathology, their median follow-up was 37.8 months (range: 18-228). Of them, the MC kept unchanged in 134 cases (62.91%); 47(22.06%) died from any unrelated cause, and 10 others evolved into malignant monoclonal gammopathy. The median clinical course of these last expanded to 60 months (range: 11-124), with an accumulated actuarial risk of 4.5% at 5 years, 15% at 10 years and 26% at 15 years.

[Diagnosis and therapeutic management of gastric lymphomas]. / Diagnóstico y manejo terapéutico de los linfomas gástricos.

PURPOSE: The optimal management of primary gastric non-Hodgkin's lymphoma (PGL) remains controversial. The purpose of this paper is to describe the histopathology, clinical behavior and management of 15 patients with PGL. PATIENTS AND METHODS: All patients were diagnosed of PGL in our Center from January 1985 to September 1995. Resection specimens were reexamined and cases were reclassified according to the concept of MALT-derived lymphoma. Age, sex, symptoms, localization, stage, treatment, complications and response were reported. RESULTS: 9 patients had low-grade B-cell lymphoma of MALT type, 5 had high-grade B-cell lymphoma (one with evidence of low-grade lymphoma of MALT type) and 1 had centroblastic-centrocytic B-cell lymphoma. Seven cases were diagnosed by endoscopy biopsies, 8 through laparatomy specimens. Stage was I in 3 cases, II1 in 5, II2 in 5, III in 0 and IV in 2. 13 patients had undergone primary gastric resection: 10 received additional chemotherapy and 1 radiotherapy. The two cases with stage IV have died: one died after surgery and one relapsed immediately after chemotherapy. After a median follow-up of 40 months, 13 patients are alive: 11 in complete response and 2 in relapse. Minor surgical complications occurred in 12 patients and major complications in 6 patients. CONCLUSIONS: Gastric resection still plays an important role in the management of early-stage PGL. Chemotherapy can be an effective therapeutic procedure in unresected or surgical high-risk patients.

[Non-Hodgkin's lymphomas. I. Clinico-biological features of 307 cases]. / Linfomas no hodgkinianos. I. Características clínico biológicas de 307 casos.

PURPOSE: To assess the clinico-biological features appearing in 307 patients with non-Hodgkin's lymphomas (NHL). PATIENTS AND METHODS: The clinical records of 338 patients diagnosed of NHL between January 1975 and December 1988 were revised in retrospect. All cases with histologic diagnosis of NHL aged over 14 years were included, and classified in accordance with the Working Formulation criteria. The following data were analysed: age, sex, first complaints, time elapsed since onset, histologic type, number of sites involved, bulky disease, anaemia, thrombocytopenia, LDH, stage, type of treatment and initial response, survival, and cause of death. The statistical evaluation was performed by actuarial analysis (Kaplan and Meier) and comparison (log-rank test) of survival. RESULTS: According to the three categories of the malignancies, the NHL were distributed into low-grade (37.8%), intermediate (36.1%) and high-grade (26.9%). The mean age of the series was 56.6 years and the M/F ratio was 1.3. Lymph node enlargement was the commonest finding; 36.4% of the patients had symptoms related with the disease, and 26.7% had bulky disease. Anaemia was present in 37.7% of the cases and thrombocytopenia in 14.3%, with similar distribution among the three grades. High LDH levels were found in 44% of the patients. At diagnosis, 85% of the patients were in advanced stages (III+IV) already. Complete response was attained in 51.1% of the cases, with median survival of 48 months. CONCLUSIONS: The clinico-evolutive data found here are similar to other reports in the literature. In one-half of the patients the cause of the first visit is lymph node enlargement. Complete remission is achieved by one out of two patients, this figure being similar for each of the histologic groups. The Working Formulation is useful in determining the different prognostic groups with respect to survival.