Bullous pyoderma gangrenosum as a predictor of hematological malignancies

Abstract: Pyoderma gangrenosum may precede, coexist or follow diverse systemic diseases. The bullous variety is usually associated with hematologic disorders. From 31 patients with pyoderma gangrenosum diagnosed in our hospital during 10 years, only 2 presented with the bullous type. One patient had previous diagnosis of chronic myeloid leukemia. Both had fast-progressive, painful lesions, and both showed an excellent response to systemic corticosteroids. In less than 3 months the lesions recurred simultaneously with the progression of chronic myeloid leukemia in one patient and with the initial diagnosis of acute myeloid leukemia in the other one. They died in less than four weeks. These findings emphasize the importance of its timely diagnosis. Cutaneous lesions could be the first predictor of an underlying disease.

El priapismo en la Leucemia Mieloide Crónica Hallazgos radiológicos en casos de Leucemia Mieloide Crónica que se presentaron con priapismo / 7 PRIAPISM IN CHRONIC MYELOID LEUKEMIA : IMAGING FINDINGS IN CASES OF CHRONIC MYELOID LEUKEMIA WHO PRESENTED WITH PRIAPISM

El priapismo es una emergencia urológica que debe ser diagnosticada y tratada apropiadamente. Específicamente en casos conocidos de leucemia el pene turgente doloroso debe hacer surgir la sospecha de priapismo de primera movida y el Doppler peneano debe ser la mera línea de modalidad de imágenes. Esta información debe ser transmitida al paciente y al departamento clínico emergentológico

Priapism is an urological emergency and must be diagnosed and treated appropriately. Specifically in known cases of leukemia painful turgid penis should raise suspicion of priapism in first hand and penile Doppler should be the first line of imaging modality. This information should be forwarded to patient and emergency department clinician

Tratamiento de la leucemia promielocítica aguda con acido transretinoico en dosis de 25 miligramos por metro cuadrado / Treatment of acute promyelocytic leukemia with acid trans dose of 25 milligrams per square meter

Se realizó un estudio observacional, descriptivo, de serie de casos, en el Hospital Esdcuela Dr. Roberto Calderón G. (HEDRCG) durante el periodo de 2002 al 2007, con el objetivo de determinar la respuesta a la administración de acido transretinoico en dosis de 25 mg/m cudrado de superficie corporal en los pacientes con diagnóstico de leucemia promielocitica aguda. Ingresados en el servicio de Heamto- ontología del Hospital en estudio. El universo lo constituyeron todos los pacientes diagnósticados con leucemia promielocitica aguda en el HEDRCG registrados en el servicio de estadísticas del Hospital. La muestra la conformaron 14 pacientes diagnósticados con leucemia promielocitica aguda en el HEDRCG durante el periodo de estudio que fueron seleccionados según criterios de inclusión y de exclusíon. No podemos dejar de mencionar que aunque nuestra muestra es poica, los estudios internacionales tampoco reportan en gran cantidad de pacientes, en la mayor parte los estudios no pasan de 25 pacientes estudiados. El 92 por ciento tenían manifestaciones de sangrado al ingreso al Hospital y la localización más frecuente fue la piel y mucosa...

Calcinosis cutis: a rare complication of chronic myeloid Leukemia

Calcinosis cutis is a term for a group of disorders in which calcium deposits are formed in the skin. It is classified into 4 major types: dystrophic, metastatic, iatrogenic and idiopathic. In all cases insoluble compounds of calcium and/or phosphate are deposited within the skin due to local/systemic factors

Fusariosis diseminada por Fusarium oxysporum en un paciente adulto con leucemia mieloide aguda y neutropenia severa febril / Disseminated fusariosis by Fusarium oxysporum in an adult patient with acute myeloid leukemia and severe febrile neutropenia

Reportamos el primer caso de fusariosis diseminada en un paciente adulto en Chile, con una neoplasia hematológica y tratamiento quimioterápico, quien evolucionó con neutropenia febril prolongada, refractaria, fuera tratado con un amplio esquema antibacteriano y desarrollara una infección multisistémica, con compromiso cutáneo, sinusal y pulmonar por Fusarium oxysporum. Cursó con refractariedad al tratamiento antifúngico con anfotericina B deoxicolato y caspofungina, utilizados en forma secuencial. El desenlace fatal de este paciente se asoció a la persistencia de la neutropenia y a la infección por un hongo filamentoso habitualmente resistente a terapia antifúngica.

Ecthyma gangrenosum: a rare cutaneous manifestation caused by pseudomonas aeruginosa without bacteraemia in a leukaemic patient--a case report.

Ecthyma gangrenosum is a rare and invasive cutaneous infection caused by Pseudomonas aeruginosa in the majority of cases, typically affecting immunocompromised patients, particularly those with neutropenia. We report a rare case of ecthyma gangrenosum in the absence of bacteraemia presenting as a solitary necrotic ulcer in a female patient with acute lymphoblastic leukaemia. A culture from the ecthyma lesion revealed the presence of Pesudomonas aeruginosa, but the results of repeated blood cultures were negative. The patient responded well to amikacin to which the isolate was susceptible in vitro. Considering high rate of mortality, early diagnosis and prompt effective treatment is mandatory.

Sarcoma granulocítico em órbita: relato de caso / Orbital granulocytic sarcoma: case report

O sarcoma granulocítico é tumor que freqüentemente aparece em pacientes portadores de leucemia mielóide aguda, podendo aparecer em diferentes regiões do corpo, incluindo a órbita. Nesta última localização, é mais freqüente em crianças e adultos jovens, com discreta predominância em pacientes do sexo masculino. Este é um caso de sarcoma granulocítico orbitário de evolução rápida, sem manifestação sistêmica associada em uma paciente de 33 anos de idade, o que o torna incomum. O surgimento do sarcoma granulocítico orbitário sem acometimento leucêmico pode ocorrer em cerca de 88 por cento dos pacientes com acometimento orbitário. A maioria dos pacientes apresenta evidências hematológicas de comprometimento sistêmico em 2 meses após a manifestação orbitária. Neste relato de caso, a paciente não apresenta acometimento sistêmico, apesar da manifestação orbitária estar presente há 30 meses. Os principais diagnósticos diferenciais do sarcoma granulocítico orbitário são o linfoma, o rabdomiossarcoma e o neuroblastoma. O diagnóstico pode ser dificultado, principalmente nos casos sem acometimento sistêmico, nos quais os exames de imagem e as manifestações clínicas pouco diferem de outras doenças. Para o diagnóstico deve-se realizar uma biópsia da lesão orbitária para análise anatomopatológica e imuno-histoquímica. O tratamento nos casos de sarcoma granulocítico orbitário sem acometimento sistêmico não é padronizado. A hipótese diagnóstica de sarcoma granulocítico orbitário deve ser aventada em casos de pacientes com tumoração orbitária mesmo que não apresentem sinais ou sintomas sistêmicos e independentes da faixa etária.

Down syndrome with transient myeloid leukemia and urological abnormality.

Among the various anomalies associated with Down syndrome, leukemia is quite common. The variant transient myeloid leukemia is seen almost exclusively in the Down syndrome patients. On the other hand, urological anomalies are infrequently found both in the Down syndrome and leukemia patients. We report a case who had the rare combination of a urological anomaly along with Down syndrome and transient myeloid leukemia.

Scopulariopsis brevicaulis infection in a patient with acute myeloid leukemia

Scopulariopsis brevicaulis is a soil fungus normally associated with onychomycosis. It causes subcutaneous infection in immunocompromised patients and is rarely isolated from blood. A case of systemic Scopulariopsis brevicaulis infection was reported in a patient with acute myeloid leukemia. The patient developed persistent fever that did not respond to wide spectrum antibiotics and amphotericin B. Scopulariopsis brevicaulis was the only pathogen isolated from blood cultures. The fever subsided with itraconazole and there was no recurrence of fungal infection with prolonged maintenance of oral itraconazole.

Acute myeloid leukemia presenting as splenic rupture.

We describe a rare case of acute myeloid leukemia presenting primarily as an acute abdomen due to spontaneous splenic rupture in a 19 years male patient. He was treated with splenectomy after failure of conservative management for splenic preservation but later succumbed to an intracerebral haemorrhage.

Mucinosis folicular generalizada / Generalized folicular mucinosis

Las lesiones de mucinosis folicular suelen localizarse en la cabeza o más raramente generalizarse a cuello, tronco y extremidades. Los hallazgos histológicos se caracterizan por la degeneración reticular del folículo pilosebáceo y el depósito de glicosaminoglicanos (mucina). Se presenta un caso de mucinosis folicular generalizada en un paciente varón de 21 años de edad. El examen reveló múltiples placas alopécicas bien definidas, eritematosas, formadas por pápulas queratósicas foliculares. Se destaca la gran extensión, el número de lesiones y la necesidad de seguimiento clínico e histológico ante la posibilidad de transformación en un linfoma cutáneo

Eritema anular centrífugo: estudo retrospectivo / Erythema annulare centrifugum: a retrospective study

FUNDAMENTO - Eritema anular centrífugo (EAC) é dermatose pouco comum, com etiologias variadas e necessidade de investigaçäo. OBJETIVOS - Estabelecer a correlaçäo clínica, epidemiológica e comparar os achados com a literatura. PACIENTES - Estudo retrospectivo de dez pacientes, realizado no ambulatório de Dermatologia da Universidade Federal do Paraná e consultório privado, no periódo de agosto de 1968 a outubro de 1993. RESULTADOS - Apesar de o diagnóstico clínico ser fácil, há dificuldade de se estabelecer a etiologia, sendo necessária investigaçäo clínica e laboratorial básica.

Prevención de mielotoxicidad en un caso de transplante medular con factor estimulante de colonias granulacito-macrófago asociado a ganciclovir / Use of GM-CSF as prophylaxis of ganciclovir-induced severe bone marrow suppression

Se informa la primera experiencia mexicana con el uso de factor estimulante de colonia granulocito-macrófago (GM-CSF) com profilaxis de la neutropenia secundaria a ganciclovir, en la prevención de la enfermedad por citomegalovirus (CMV) en un paciente CMV sero-positivo con leucemia mieloide aguda en primera remisión, trasplantando con donador HLA idéntico y CMV sero-positivo. La toma de injerto ocurrió el día 14. Se inició ganciclovir 5 mg/kg/3 veces por semana) en el día 35 acompañandose de toxicidad medular secundaria 28 días después con neutropenia grave que remitió de manera espontánea posterior a la suspensión del mismo. A fin de concluir el esquema de profilaxis, se inició GM-CSF a dosis de 300 mg/kg/día concomitante al ganciclovir a dosis de 5 mg/kg/día con lo cual fue posible conluir tratamiento sin que se reindujera toxicidad medular. No hubo evidencia de enfermedad de injerto contra huésped ni de infección por CMV. La evolución del paciente fue satisfactoria durante un año, posterior al cual, presentó recaída de su enfermedad de base mueriendo por complicaciones secundarias a leucemia

Unsuspected gastric granulocytic sarcoma in a patient with myelodysplastic syndrome

Granulocytic sarcoma (GS) is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. Most GS reported in large series were not associated with overt acute myelogenous leukemia. Gastric perforation occurred during prednisolone therapy in a 72-year-old Japanese male with a four-month history of a myelofibrosis-like state. Subtotal gastrectomy was performed for a suspected gastric ulcer perforation. Gastric histologic, immunohistochemical and cytochemical examination revealed diffuse infiltration by sheets of myeloblasts and promyelocytes with scant or moderately abundant cytoplasm including a few eosinophilic myelocytes. Bone marrow study done in one month after the operation disclosed refractory anemia with excess of blasts (RAEB). Leukemic transformation occurred two months later, and a subcutaneous tumor appeared on the forehead. The forehead tumor predominantly consisted of myeloblasts without evidence of maturation. Both the stomach and forehead tumors were examined immunohistochemically with a panel of monoclonal antibodies (LCA, L26, MT1, UCHL1, OPD4, LN-1, LN-2, LN-3, MB1, Leu-M1, PM) and polyclonal antibodies (lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin, S-100 protein, lactoferrin), as well as naphthol-ASD-chloroacetate esterase staining to investigate and characterize the reliable marks for GS, and the patient was diagnosed as GS. We found that gastric GS may occur in a myelofibrosis-like state followed by RAEB of myelodysplastic syndrome and that naphthol-ASD-chloroacetate esterase staining and immunohistochemical detection of MT1, lysozyme, and alpha 1-antitrypsin were the most reliable markers for confirming the diagnosis of GS.