Dermatosis neutrofílica de las manos y rostro posterior a mordedura de perro: reporte de caso y revisión de la literatura / Neutrophilic dermatosis of the hands and face after dog bite: case report and literature review

La Dermatosis neutrofílica de las manos es consi-derada una variante localizada acral del Síndrome de Sweet, más frecuente en mujeres y principal-mente asociada a enfermedades hematológicas. Las lesiones aparecen como pápulas, vesículas, nó-dulos, placas, úlceras y ampollas, principalmente en el dorso de las manos. Aproximadamente la mi-tad de los pacientes presenta fenómeno de patergia como factor desencadenante.En el presente caso clínico se describe una derma-tosis neutrofílica de las manos posterior a morde-dura de perro, asociado a mielofibrosis primaria y desarrollo de lesiones faciales.

Neutrophilic dermatosis of the hands is conside-red an acral localized variant of Sweet Syndrome, more frequent in women and mainly associated with hematological diseases. The lesions appear as papules, vesicles, nodules, plaques, ulcers, and blisters, mainly on the back of the hands. Appro-ximately half of the patients present a phenome-non of pathergy as a triggering factor. Herein we describe a case of neutrophilic dermatosis of the hands after a dog bite, associated with primary myelofibrosis and development of facial lesions.

Male with myelofibrosis and ulceronecrotic lesions

SUMMARY Granulocytic sarcoma also called myeloid sarcoma is an extramedullary tumour of immature granulocytic cells. It is a rare entity, and mostly accompanied by acute myeloid leukaemia. It is observed during the course of myeloproliferative disorders especially in chronic myeloid leukaemia and myelodysplastic syndromes. Here, we report a case of a 60-year-old male with past history of myelofibrosis admitted to the emergency room due ulceronecrotic lesions, fever and dysphagia. We emphasize the importance of recognizing this entity and its severity.

RESUMO O sarcoma granulocítico, também chamado de sarcoma mieloide, é um tumor extramedular de células granulocíticas ¡maturas. É uma entidade rara, e principalmente acompanhada de leucemia mieloide aguda. É observado durante o transtorno mieloproliferativo, especialmente na leucemia mieloide crónica e síndromes mielodisplásicas. Aqui, relatamos um caso de um homem de 60 anos com antecedente de mielofibrose admitida na sala de emergência devido a lesões ulceronecróticas, febre e disfagia. Enfatizamos a importância de reconhecer essa entidade e sua gravidade.

Janus Activated Kinase inhibition in Myelofibrosis.

Janus Activated Kinase (JAK) 2 plays an important role in the pathogenesis of myelofibrosis (MF). Ruxolitinib (INCB018424, Jakafi) is a potent dual JAK1 and JAK2 inhibitor. In November 2011, it became approved by the US FDA for the treatment of intermediate or high-risk MF. This review shall outline the role of Ruxolitinib in the current management of MF and its potential future.

JAK2-positive Philadelphia-negative myeloproliferative neoplasms.

The recent discovery of the JAK2 mutations has rekindled interest in the approach to classic BCR/ABL-negative myeloproliferative neoplasms (MPNs) in terms of both diagnostic evaluation and treatment. However, additional clinical, laboratory and histological parameters play a key role to allow diagnosis and subclassification, regardless of whether JAK2 V617F mutation is present or not. Here are two cases which incidentally presented with splenomegaly and moderate leukocytosis, and were diagnosed as MPN-primary myelofibrosis (PMF) in prefibrotic phase and polycythemia vera (PV), respectively, using revised World Health Organization (WHO) 2008 criteria.

Histological and clinical evolution patterns of chronic myelocytic leukemia.

Trephine biopsies of 101 chronic myelocytic leukaemia (CML) patients were analysed to study the relationship between initial and subsequent histological features vis-a-vis clinical behaviour of the disease. The patients with blast crisis at presentation were excluded. At diagnosis 62 (61.4%) patients revealed granulocytic-megakaryocytic (gran-meg) proliferation whereas granulocytic (gran) proliferation was found in 39 (38.6%) patients. Gran pattern at diagnosis was associated with shorter survival and early evolution into blast crisis (36.8%) in 12 months, although the difference in the total incidence of blast crisis between the two histological groups was not statistically significant. Myelofibrosis was detected in more number of cases on follow up (89.1%) as compared to the initial biopsies (80.2%). However myelofibrosis did not correlate with initial cellular composition, overall survival or the phase of CML (P > 0.05). Transition from one histological type to another was observed in 15 out of 60 (25%) cases while remaining in the chronic phase.

Acute myelofibrosis.

Acute myelofibrosis is a rare disorder. Five such cases have been diagnosed out of 3,149 Bone Marrow (B.M) Aspirations and Trephine Biopsies studied over a period of eight and a half years. Four out of five patients were males and the other female. Their age ranged from 14-71 years. Neither splenomegaly not red cell poikilocytosis (tear drop cells) were prominant features. B.M. trephine biopsy showed increase of atypical megakaryocytes/megakaryoblasts and marked fibrosis. There was rapid downhill clinical course of the disease in all cases.

Relative efficacy of bone marrow trephine biopsy sections as compared to trephine imprints and aspiration smears in routine hematological practice.

The relative efficacy of trephine sections, trephine imprints and aspiration smears in yielding diagnostic and additional information was compared in 767 sets of bone marrow samples. Trephine sections were diagnostic in significantly more cases as compared to trephine imprints and aspiration smears (P < 0.001). Additional information was obtained in 326 trephine sections which was not available from trephine imprints and aspiration smears. Significantly more number of trephine sections provided diagnosis in case of dry tap/scanty material, for assessment of lymphoma-tumour infiltration, cellularity, Perl's reaction, megakaryocyte density and proliferating cell lines in myeloproliferative disorders. Fibrosis of bone marrow, pattern of bone marrow involvement and topographical alterations were appreciable only on trephine sections. The differential counts done on trephine imprints and aspiration smears correlated well and cytomorphological characterisation of immature cells (blasts and promyelocytes) could be done on these two preparations. Although trephine sections provide maximum information, all three preparations were found complementing each other and should be evaluated simultaneously for complete bone marrow interpretation.

Critérios morfológicos para el diagnóstico de entidades mieloproliferativas / Morphologic criterium for the diagnosis of myeloproliferative entities

Se estudiaron 212 biopsias de procesos mieloproliferativos con definidos criterios histopatológicos para el diagnóstico de las siguientes entidades: mielosis megacariocítica, policitemia vera, leucemia granulocítica crónica y leucemia megacreioblástica. Todas tienen en común una proliferación megacariocítica aumentada y/o anormal pero diferente en cada una de ellas. Además se considera la interrelación entre las mismas, la incidência de crisis blástica y su evolución mielofibrótica, o sea, la mielofibrosis no es reconocida como una entidad independiente sino como una posibilidad final común; también y en relación a ello se prefiere reemplazar el término metaplasia mieloide angógena por el de mielofibrosis (generalmente presente en una mielosis megacariocística granulocítica crónica) con metaplasia mieloide