RESUMO
Therapy-related myelodysplastic syndrome (t-MDS) and therapy-related acute myelogenous leukemia (t-AML) in patients with acute promyelocytic leukemia (APL) are rare events. The cumulative exposure to chemotherapy with alkylating agents and topoisomerase II inhibitors is associated with t-AML that may develop any time after the completion of the treatment. We report the case of an acquired AML who previously received therapy for APL, after two years of being diagnosed. The diagnosis was established by morphologic findings, membrane markers, cytogenetic studies, and fluorescence in situ hybridization (FISH). To our knowledge this is the first documented case in Puerto Rico of a patient with APL that developed a t-AML without the characteristic chromosomal and morphologic findings of APL.
Assuntos
Leucemia Mieloide Aguda/etiologia , Leucemia Promielocítica Aguda/tratamento farmacológico , Segunda Neoplasia Primária/etiologia , Translocação Genética , Adulto , Antineoplásicos Alquilantes/administração & dosagem , Antineoplásicos Alquilantes/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Cromossomos Humanos Par 15/ultraestrutura , Cromossomos Humanos Par 17/ultraestrutura , Cromossomos Humanos Par 21/ultraestrutura , Cromossomos Humanos Par 3/ultraestrutura , Cromossomos Humanos Par 7 , Citarabina/administração & dosagem , Daunorrubicina/administração & dosagem , Daunorrubicina/efeitos adversos , Sinergismo Farmacológico , Evolução Fatal , Humanos , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/genética , Leucemia Promielocítica Aguda/genética , Masculino , Mercaptopurina/administração & dosagem , Metotrexato/administração & dosagem , Metotrexato/efeitos adversos , Mitoxantrona/administração & dosagem , Mitoxantrona/efeitos adversos , Monossomia , Segunda Neoplasia Primária/genética , Tretinoína/administração & dosagemRESUMO
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.
Assuntos
Leucemia Monocítica Aguda/patologia , Infiltração Leucêmica , Pele/patologia , Feminino , Humanos , Leucemia Monocítica Aguda/terapia , Pessoa de Meia-IdadeRESUMO
The following case illustrates an ileal perforation and reactive hemophagocytic syndrome (RHS) resulting from disseminated histoplasmosis in a patient with Human Immunodeficiency Virus (HIV) from Puerto Rico. Although the diagnosis was established by histopathologic findings and a positive bone marrow culture, Histoplasma capsulatum-specific real-time Polymerase Chain Reaction (PCR) allowed to confirm the diagnosis from formalin-fixed, paraffin-embedded tissue. Interestingly, the Histoplasma antigens in both serum and urine samples were falsely negative. Amphotericin B lipid complex (Abelcet), followed by oral itraconazole, led to a successful response and resolution of symptoms. A short review of the clinical signs and symptoms, diagnostic tests, and therapeutic options for disseminated histoplasmosis is done, with emphasis on the role of Histoplasma-specific real-time PCR as a molecular diagnostic tool and the efficacy of treatment with one of the lipid formulations of amphotericin B.
Assuntos
Anfotericina B/uso terapêutico , Antifúngicos/uso terapêutico , Histoplasma/isolamento & purificação , Histoplasmose , Perfuração Intestinal/complicações , Linfo-Histiocitose Hemofagocítica/complicações , Fosfatidilcolinas/uso terapêutico , Fosfatidilgliceróis/uso terapêutico , Infecções Oportunistas Relacionadas com a AIDS/complicações , Infecções Oportunistas Relacionadas com a AIDS/diagnóstico , Infecções Oportunistas Relacionadas com a AIDS/tratamento farmacológico , Infecções Oportunistas Relacionadas com a AIDS/microbiologia , Adulto , Anfotericina B/administração & dosagem , Antifúngicos/administração & dosagem , Combinação de Medicamentos , Infecções por HIV/complicações , Infecções por HIV/virologia , HIV-1/isolamento & purificação , Histoplasma/genética , Histoplasmose/complicações , Histoplasmose/diagnóstico , Histoplasmose/tratamento farmacológico , Histoplasmose/microbiologia , Humanos , Íleo/patologia , Masculino , Fosfatidilcolinas/administração & dosagem , Fosfatidilgliceróis/administração & dosagem , Porto Rico , Resultado do TratamentoRESUMO
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
Assuntos
Humanos , Adolescente , Feminino , Células Neoplásicas Circulantes/patologia , Rabdomiossarcoma/sangue , Neoplasias Vulvares/sangue , Biópsia , Medula Óssea/patologia , Pancitopenia/sangue , Pancitopenia/patologia , Rabdomiossarcoma/patologia , Neoplasias Vulvares/patologia , Vulva/patologiaRESUMO
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