Aleukemic leukemia cutis preceding acute monocytic leukemia: a case report.

Aleukemic leukemia cutis is an extremely rare clinical presentation in patients who eventually develop acute leukemia, usually of monocytic lineage. This condition is associated with a very poor prognosis and is often difficult to diagnose. We report a case of a 33 years old female with leukemia cutis preceding the onset of acute monocytic leukemia by four months. The patient received induction and consolidation chemotherapy followed by allogeneic bone marrow transplant and has been free of disease for six years. To our knowledge, this is the first documented case in Puerto Rico with the diagnosis of leukemia cutis preceding acute monocytic leukemia.

Dose escalation studies of cytarabine, daunorubicin, and etoposide with and without multidrug resistance modulation with PSC-833 in untreated adults with acute myeloid leukemia younger than 60 years: final induction results of Cancer and Leukemia Group B Study 9621.

PURPOSE: P-glycoprotein (Pgp) is strongly inhibited by PSC-833. A chemotherapy dose-escalation study was performed with PSC-833 in patients younger than 60 years with untreated acute myeloid leukemia. Clinical rather than pharmacokinetic end points were used to develop two induction therapies containing drugs susceptible to Pgp-mediated efflux and associated with comparable toxicities at the maximum-tolerated doses. PATIENTS AND METHODS: A total of 410 patients were enrolled. Fifteen induction regimens containing variable doses of daunorubicin (DNR) and etoposide (ETOP) and fixed doses of cytarabine were evaluated with (ADEP) or without (ADE) a fixed dose of PSC-833. RESULTS: Doses selected for phase III testing were DNR 90 mg/m(2) and ETOP 100 mg/m(2) in ADE, and DNR and ETOP each 40 mg/m(2) in ADEP. Intolerable mucosal toxicity occurred at higher doses of ADEP. Although the design of this study precludes direct comparisons, there was an apparent advantage for receiving ADEP with respect to disease-free and overall survival in patients < or = 45 years old, despite the significantly lower doses of DNR and ETOP given in ADEP compared with ADE. CONCLUSION: A large clinical data set was used to develop induction regimens containing two drugs susceptible to Pgp-mediated efflux, with and without an inhibitor of Pgp function. The chosen doses have comparable antileukemia activity and toxicity, making them suitable for use in a phase III comparative study of induction chemotherapy for patients with acute myeloid leukemia younger than 60 years. That trial will also clarify whether patients < or = 45 years old are especially likely to benefit from Pgp inhibition during induction therapy.

Lineage specific treatment of adult patients with acute lymphoblastic leukemia in first remission with anti-B4-blocked ricin or high-dose cytarabine: Cancer and Leukemia Group B Study 9311.

BACKGROUND: Anti-B4-blocked ricin is an immunotoxin comprised of an anti-CD19 murine monoclonal antibody (B4) conjugated to blocked ricin, which has cytotoxic activity in patients with lymphoid malignancies. METHODS: Adults with untreated acute lymphoblastic leukemia (ALL) were treated with a previously developed and tested chemotherapeutic regimen. Patients with CD19 positive ALL were given anti-B4-blocked ricin as 2 7-day continuous infusions 1 week apart. Patients with CD19 negative ALL received high-dose cytarabine. Serial polymerase chain reaction (PCR) assays of BCR-ABL, immunoglobulin heavy chain (IGH), and T-cell receptor (TCR) genes were used to measure the impact of lineage specific intensification treatment on minimal residual disease. RESULTS: Eighty-two adults were enrolled, and 78 were eligible. The median age was 34 years (range, 17-81 years). Sixty-six patients (85%) achieved complete remission. Forty-six patients received the anti-B4-blocked ricin, which generally was well tolerated; 80% were able to receive both courses. The most common toxicity was asymptomatic transient elevation of liver function tests in 72% of patients. Lymphopenia occurred in 46% of patients. Two patients developed antibodies to the anti-B4-blocked ricin. Molecular monitoring before and after the experimental course of intensification did not show a consistent change in the number of leukemia cells remaining, and the immediate posttreatment PCR studies did not correlate with remission duration. CONCLUSIONS: Intensification therapy with anti-B4-blocked ricin is feasible for patients with CD19 positive ALL, although there is little evidence of an additional clinical benefit from the anti-B4-blocked ricin. Cancer 2003;97:1471-80.

Carcinocythemia in a patient with rhabdomyosarcoma

Solid tumor cells are rarely seen in peripheral blood smears. When this occurs the term carcinocythemia is used. This report describes an 18 years old female who presented with a painless lump in the labia majora associated with pancytopenia. Tumor cells were identified int he peripheral blood smear and the bone marrow aspirate showed a predominant population of small round vacuolated primitive cells, many of which formed clumps of varying sizes. Biopsies of the vulvar mass and bone marrow were interpreted as alveolar rhabdomyosarcoma. Review of the literature revealed 12 previously reported cases in whom carcinocythemia had been documented; rhabdomyosarcoma was the specific cell type involved in only two of these. The median time between detection of the leukemic phase of the tumor and death was 8.5 weeks, reflecting the fact that carcinocythemia, when it occurs, represents the terminal event of the disease. To our knowledge, our case is the third well documented case of rhabdomyosarcoma in leukemic phase so far reported. The clinical evolution as well as the management of this patient will be described in detail along with a review of the partinent available literature

Adult idiopathic thrombocytopenic purpura

Fifty three adult patients with idiopathic thrombocytopenic purpura (ITP) were diagnosed at the University District Hospital (UDH) during the last 10 years (1976-1986). There were 33 females (62%) and 20 males (38%). All the patients were over 15 years old with a mean age of 3o years. Only 13 patients gave a history of a viral syndrome preceding the onset of symptoms. The most frequent chief complaint was petechiae and/or ecchymotic lesions. The mean initial platelet count was 10,000/mm. Bone marrow aspiration showed an increased number of megakaryocytes without platelet production in 49 patients (86%). Of the fifty three patients, 37 (70%) had a complete response to steroids. Of those not responding to steroids, 15 underwent splenectomy and 11 (73%) achieved a permanent response. Immunosupresion was prescribed in 4 patients with a 50% complete response

Coexistent polycythemia vera and smoldering multiple myeloma: report of a case and review of the literature

Polycythemia vera is a myeloproliferative disorder rarely associated with other hematologic malignancies be sides leukemia. Very seldon, an immunoglobulin abnormality may be seen without clinical evidence of a coexistent plasma cell disorder. The association of polycytemia vera and multiple myeloma is extremely rare and in only six of the previously reported cases, both diagnoses were made simultaneously. We report the case of an asymptomatic 58 years old hipertensive male patient who during a routine lalboratory evaluation was found to have thrombocytosis (931,000/mm3) and mild leukocytosis (14,300/mm3). Bone marrow aspiration revealed an increased number of immature plasma cells with absent iron stores. Further laboratory studies were as follows: hemoglobin level 19.6gm/dl; white blood count 19,200/mm3 and platelet count 486,000/mm3. The total serum proteins were 9.1 gm/dl with a serum protein electrophoresis showing hyper gamma-globulinemia (3.2 gm/dl). Immunoglobulin quantitation showed an IgA of 3 gm/dl. The total red cell mass was 38.6cc/Kg and the arterial oxygen saturation was 93%. The leucocyte alkaline phosphatase test score was 271. These studies suggested the coexistence of two diseases: polycythemia vera and multiple mueloma. A detailed description of the clincial manifestations and the laboratory studies of this case as well as a reviwe of the pertinent literature form the basis of this report

Chronic lymphocytic leukemia: a study of 53 patients and review of the literature

La leucemia crónica linfocítica tiene un curso clínico muy variable. Es el tipo de leucemia más frecuente en el hemisferio occidental, sin embargo, hemos tenido la oportunidad de evaluar y seguir solo 53 pacientes durante los últimos 14 años. La mayoría de los pacientes eran de origen caucásico, con edad promedio de 61 años y predominio en el varón a razón de 3:2.3. Ocho de los pacientes presentaron con hipogamaglobulinemia y cinco con la prueba de Coomb's positiva. Solo un paciente presentó manifestaciones clínicas de hemólisis y no se observaron otras manifestaciones inmunológicas. Entre las complicaciones se informaron: una fractura patológica, una neoplasia secundaria y un paciente con hiperuricemia. El estadío clínico se llevó a cabo siguiendo el esquema de Rai: 0:12 (23%), I: 11(21%), II: 17(32%), III: 5(9%), y IV: 8(15%). Las indicaciones para tratamiento en la mayoría de los casos fueron la presencia de linfoadenopatías y esplenomegalia. Todos los pacientes recibieron chlorambucil con o sin prednisona y un 66% de éstos obtuvieron respuestas a la quimioterapia. Las peores respuestas se observaron en los pacientes en estadió IV. Cuatro pacientes fallecieron durante los primeros 6 meses después de hecho el diagnóstico, uno en estadío III y tres en estadío IV. Unos 22 pacientes han tenido un seguimiento mínimo por 5 años, de los cuales: 7(31%) fallecieron, 3 en estadío III y 4 en estadío IV. Este estudio sugiere que tal vez, una terapia más agresiva debe ser ofrecida inicialmente a aquellos pacientes que se presentan en estadíos avanzados. A pesar del reducido número de nuestra serie los resultados comparan favorablemente con los informados por otros autores

Multiple myeloma terminating in acute leukemia: report of two cases and review of the literature

La presentación simultánea de mieloma múltiple y otras enfermedades hematológicas en un mismo paciente, se observa en muy rar as ocasiones. Se han publicado muy pocos casos en la literatura en que coexiste mieloma múltiple y leucemia aguda. En la mayoría de estos casos los pacientes presentaron la leucemia después de haber recibido tratamiento prolongado con agentes alquilantes, razón por la cual se ha implicado a estos como posible factor etiológico causal de la leucemia. Durante los últimos 14 años hemos seguido 141 pacientes con mieloma múltiple en el Hospittal Universitario, de los cuales 3 (2.1%) presentaron posteriormente leucemia aguda. Dos pacientes eran mujeres de 48 y 49 años y uno varón de 33 años. El diagnóstico de mieloma múltiple se estableció por la presencia de un pico protéico monoclonal en suero, lesiones óseas líticas y la presencia de una plasmacitosis inmafuera en la méfuela ósea. Los tres pacientes fueron tratados con agentes alquilantes por 2,9 y 10 años respectivamente antes del inicio de la leucemia. El evento inicial en dos de los casos fue trombocitopenia, alteraciones morfológicas de las plaquetas en uno de los pacientes y la presencia de células inmafueras en la sangre periférica. La transformación en los tres casos fue a leucemia aguda mielógena. Dos de los pacientes recibieron terapia antileucémica fuerante 9 y 11 meses respectivamente. El cuadro clínico de los tres pacientes se describe en detalle y se revisa la literatura pertinente