Waldenström's macroglobulinemia complicated with acute myeloid leukemia. Report of a case and review of the literature.

The evolution of Waldenström's macroglobulinemia (WM) into chronic or acute myeloid leukemia (AML) is a rare event. Most of these cases have occurred after treatment with alkylating agents. We herein report a case of WM terminating in an acute myelomonocytic leukemia after treatment with prednimustine and chlorambucil and present a review of the literature.

[Acute myeloid leukemia in those over 70 years of age. Experience using low-dose ara-C treatment]. / Leucemia mieloide aguda en mayores de 70 años. Experiencia utilizando tratamiento con Ara-C a bajas dosis.

PURPOSE: The treatment of elderly patients with acute myeloid leukaemia (AML) remains controversial. We present the results of the treatment of a group of patients aged above 70 years with AML diagnosed in our Hospital since 1990. PATIENTS AND METHODS: We have studied retrospectively the cases of AML in patients older than 70 years diagnosed in our Service since January 1990 to June 1996. Induction treatment was performed, in all cases but one, with two cycles of Ara-C 10 mg/m2/12 h s.c. for 21 days and after haematological recuperation, if complete remission had been achieved, monthly maintenance treatment with Ara-C (25 mg/m2/12 h oral x 5 days), prednisone (40 mg/m2/day x 5 days) y vincristine (1 mg/m2 i.v. x 1 day) was begun. RESULTS: During the period of study 48 patients with AML have been diagnosed in our Service, among them 22 (45.8%) were older than 70 years. One of them could not be considered for the study as not all data from him could be compiled. Among the other 21 patients 5 presented previous haematological processes (4 myelodysplastic syndrome and 1 Waldenström's macroglobulinemia). Initial diagnosis according to FAB classification for AML was as follows: 7 M1, 6 M2, 4 M4, 2 M5 and 2 M6. From these 21 patients 2 received no treatment due to rapid progression and death, among the other 19, one was directly treated with a modification of the maintenance treatment with vincristine and prednisone without response (survival 2 months). The other 18 patients were treated with low-dose Ara-C (described above), among them 3 (16.7%) were not evaluable as they did not finish the first cycle of induction treatment; 8 (44.4%) showed no response; 2 (11.1%) achieved partial remission and 5 (27.8%) complete remission. One patient did not show any response after two cycles of low-dose Ara-C but she obtained complete remission when treated with Ara-C and idaurubicin. Overall mean survival was 5.7 months (median 2; 95% confidence interval 1.6-9.8 months). In the group of patients treated with low-dose Ara-C mean survival was 6.6 months (median 3.5; 95% confidence interval 1.9-11.2 months). CONCLUSION: We consider that the treatment with low-dose Ara-C is a valid option in the treatment of elderly patients (aged 70 or above) with AML because 28% complete remissions can be achieved, specially in those ones in which other more aggressive treatments are not possible.

[T-cell-rich B-cell lymphoma. Presentation of 3 cases]. / Linfoma B rico en células T. Presentación de tres casos.

T-cell-rich B-cell lymphoma (TBL) is a rare entity not included in the classical classifications of non-Hodgkin's lymphomas (NHL), presenting usually at diagnosis in advanced stages and with extranodal involvement. Its differential diagnosis, based on immunophenotyping technics, include other entities with different treatment and prognosis, mainly lymphocytic-predominance Hodgkin disease and peripheral T-cell lymphoma; this differential diagnosis has importance in patient's survival. We present 3 patients, two males (one of them with HIV infection) and one female, aged 38, 60 and 64 respectively, diagnosed as having TBL. The two former patients presented advanced stages at diagnosis. None of them was initially diagnosed as having TBL, the diagnosis were immunoblastic NHL in the first patient, peripheral T-cell lymphoma in the second and mixed cellularity Hodgkin's disease in the third one. The first patient was not treated due to rapid worsening and early death of septic shock; the other two cases were treated with COP-BLAM/IMVP-16 protocol achieving partial and complete remission respectively after 6 cycles of treatment. Comments about clinical, pathological and differential diagnostic aspects are made.

A necrotic soft-tissue lesion due to Corynebacterium urealyticum in a neutropenic child.

Corynebacterium urealyticum has been associated mainly with infections of the urinary tract. Other infections due to this organism are highly unusual. We report what we believe is the first case of necrotic infection of soft tissue due to C. urealyticum in a neutropenic child who was previously treated with chemotherapy. The infection was cured when the patient was treated with vancomycin and surgical debridement. The increase in the number of neutrophils may also have contributed to the patient's recovery.

[Comparative study of the stability of oral anticoagulant treatments (warfarin vs acenocoumarol)]. / Estudio comparativo de la estabilidad del tratamiento anticoagulante oral (warfarina versus acenocumarol).

PURPOSE: To compare the stability of the effect of two oral anticoagulants, one of them (acenocoumarol) with a short half life and the other one with a long half life (warfarin) in patients in the stable phase of treatment (at least 2 months with treatment before entering the study). PATIENTS AND METHODS: During a year period (January-December 1993) a comparative study of two groups of 53 patients each was performed: group 1 patients were treated with warfarin and group 2 with acenocoumarol. Both groups were paired with respect to age, sex, diagnosis for anticoagulant therapy and desired therapeutic range (INR 3-4.5). The mean value of controls per patient, the dosage changes, the evolutive controls and the incidence of haemorrhagic and thromboembolic episodes were studied. RESULTS: The controls performed in group 1 were 728 in total with a mean value of 13.74 per patient and 800 in group 2 with a mean value of 15.09 per patient. A change in the dosage was performed in 214 controls in patients of group 1 and in 269 of group 2. Seventeen patients had 38 haemorrhagic episodes (2 major and 36 minor) in group 1, and 6 of group 2 had 20 episodes (2 major and 18 minor). Significant differences were observed in the mean value of controls (p = 0.04), the evolutive controls (p < 0.001), the global incidence of haemorrhages (p = 0.008) and incidence of minor ones (p = 0.006). No significant differences in dosage were observed. In both groups no thromboembolic episodes during the period of study were reported. CONCLUSIONS: Anticoagulant treatment with warfarin is more stable than with acenocoumarol. The total controls and the mean value of controls per patient are decreased. Nevertheless with warfarin we have observed a greater incidence of haemorrhagic episodes.

[Primary testicular lymphoma. Report of two cases]. / Linfoma testicular primario. Presentación de dos casos.

Primary testicular lymphoma (PTL) is the most frequently diagnosed testicular tumor in men over 60 years, in spite of this circumstance it is a rare process. Two cases of PTL are reported, the first one in a child and the second in an adult. Both cases were intermediate grade lymphoma and had low stage (IEA), presenting initially as an enlargement of the testicle as the only symptom, the second case presented involvement by contiguity of the abdominal muscles. Immunohistological markers showed T nature in the first case, and B in the second. The treatment applied in both cases was orchiectomy and systemic chemotherapy using COP-BLAM/IMVP-16, in the child prophylaxis of the central nervous system using methotrexate was made. Response to treatment was good, the first patient achieved complete remission and his survival at present is 24 months, the second patient died 5 month after diagnosis due to stroke without having completed chemotherapy but with an important reduction of the tumoral mass. A review of the literature on clinical, diagnostic and therapeutic issues is made.

[Treatment with oral anticoagulants (acenocoumarol): influence of the initial doses in the incidence of hemorrhagic and thromboembolic episodes]. / Tratamiento con anticoagulantes orales (acenocumarol): influencia de la dosis inicial en la incidencia de episodios hemorrágicos y tromboembólicos.

PURPOSE: To compare two initial doses of oral anticoagulant (acenocoumarin) studying the haemorrhagic and thromboembolic episodes occurred during the first month of treatment, the mean time and necessary controls until achievement of the desired level of anticoagulation. PATIENTS AND METHODS: From january 1992 to december 1993; a comparative study of two groups of patients was performed: group 1, compiling 129 patients chosen at random and retrospectively, who begun oral anticoagulant treatment with 4 daily mg of acenocoumarin; and group 2, compiling 129 patients chosen prospectively, who begun with 2 mg daily. In both groups the mean time and the number of controls performed until achieving the desired level of anticoagulation were analyzed, as well as the haemorrhagic episodes occurred during the first month of treatment, their severity (classified into major and minor ones), the level of anticoagulation when they occurred and their possible causes. In the same way the thromboembolic processes occurred during that period in both groups were studied. RESULTS: The mean time necessary to achieve the desired level of anticoagulation was 3.8 days in group 1 and 6.3 in group 2; the mean number of controls performed in group 1 was 1.2 and in group 2 it was 1.8. We have observed 19 haemorrhagic episodes, 15 in group 1 (4 minor and 11 major); and 4 in group 2 (2 minor and 2 major). We have found significant differences with respect to the mean time (p < 0.01), number of controls (p < 0.01) and incidence of hemorrhages (p = 0.017) between groups 1 and 2. One thromboembolic episode was registered in each group: in group 1 a deep venous thrombosis and in group 2 a stroke. CONCLUSION: The initial daily doses of acenocoumarin of 2 mg is as effective as the 4 mg one in the prevention of thromboembolic episodes, with a significant reduction in the number of haemorrhages observed during the first month of treatment. However this produces a prolongation in the necessary mean time and more number of controls performed until the achievement of the desired level of anticoagulation.

[Human recombinant erythropoietin in the treatment of myelodysplastic syndromes anemia. Meta-analytic study]. / Eritropoyetina recombinante humana en el tratamiento de la anemia de los síndromes mielodisplásicos. Estudio metaanalítico.

PURPOSE: To study the usefulness of different published epidemiological and analytic parameters to decide the treatment with human recombinant erythropoietin (rHuEPO) of anaemic patients with myelodysplastic syndromes (MDS). PATIENTS AND METHODS: We have revised 10 published series compiling 115 patients, studying age, sex, initial diagnosis, route of administration and posology, criteria of response, duration of the study, dosis with the response was obtained, response according to initial diagnosis, duration of responses, and effect of the treatment on other hematopoietic series. We have made a comparison between responders and non-responders based on epidemiological and analytical parameters. RESULTS: We have compiled 115 patients with a rate of global response of 23.5%. We have not found significative differences between the route (s.c. or i.v.) or frequency of administration, however the number of responses was higher when rHuEPO was administered three times weekly. A great variability in the criteria of response was observed among the different studies. Most of studies have a duration of three months but we have observed significative differences in the number of responses when the study is longer. We have not found significative differences between responders and non-responders with respect to age, sex, used dosis, transfusional dependency and degree of transfusional dependency, basal serum erythropoietin, time since diagnosis, transfusional period, haemoglobin level among non-transfusion dependent patients and haemoglobin level among transfusion dependent patients. We have found significative differences with respect to initial diagnosis, a higher rate of responses was observed in the refractory anaemia with excess of blasts (RAEB) group. We have not found a higher rate of transformations into acute myeloid leukaemia (AML) among these patients. The effects of the treatment on other haematopoietic series can be considered as anecdotical. CONCLUSION: The different epidemiological and analytic parameters published up to now are not useful in the decision of including an anaemic patient with MDS in the treatment with rHuEPO. Those patients with RAEB can be benefited with the treatment with rHuEPO. The concomitant use of other cytokines could improve these results.

[Chronic neutrophilic leukemia associated with myeloma. Simultaneous presentation]. / Leucemia neutrofílica crónica asociada a mieloma. Presentación simultánea.

A case of chronic neutrophilic leukaemia associated with multiple myeloma is reported. The patient had a 6 months history of bruising and weight loss, and showed mature neutrophilic leukocytosis, hepatosplenomegaly, high neutrophil alkaline phosphatase score, hyperuricaemia, neutrophils with pseudotoxic granulation and scarce Döhle bodies; moreover, a monoclonal IgG lambda was detected amounting 57.3 g/L. The bone marrow was grossly hypercellular with marked myeloid hyperplasia and aggregates of immature plasma cells. After treatment during 1 year with melphalan and prednisone she is well, although persisting with neutrophilic leukocytosis, slight splenomegaly, and the monoclonal IgG decreased to 25.8 g/L.

Phenytoin-induced hemocytophagic histiocytosis indistinguishable from malignant histiocytosis.

We have reported a case of phenytoin-induced hemocytophagic histiocytosis indistinguishable on clinical and histopathologic grounds from malignant histiocytosis. We emphasize the need to investigate for microbiologic causes and drug ingestion, even if the diagnosis of malignant histiocytosis is plausible. We think that reactive and malignant histiocytosis are not really two distinct entities with different etiologies, but a continuum of host responses to several insults with different degrees of aggressiveness depending on the host immune status.