Linfoma de Hodgkin: neuronopatía sensitiva y autonómica como manifestación paraneoplásica / Hodgkin's lymphoma: sensitive and autonomic neuropa thy as a paraneoplastic manifestation

Resumen El linfoma de Hodgkin (LH) comprende un grupo heterogéneo de neoplasias linfoides cuyo origen radica en linfocitos B. Las manifestaciones neurológicas de dicha enfermedad son infrecuentes, pudiendo tener su origen por invasión directa de las células neoplásicas en el sistema nervioso, o indirectamente, a través de síndromes paraneoplásicos o como complicación del tratamiento. Dentro de los síndromes neurológicos paraneoplásicos que afectan a pacientes con LH, la degeneración cerebelosa paraneoplásica es la más frecuente. Otros reportados con menor frecuencia en series de casos o casos aislados incluyen encefalitis límbica, neuronopatía sensitiva, motora y autonómica. Estos pueden ser la manifestación inicial de la enfer medad neoplásica, y la falta de conocimiento de dicha asociación puede retrasar el diagnóstico, con inicio tardío del tratamiento y peor pronóstico. Reportamos el caso de una mujer con LH que presentó al inicio de su enfermedad neuronopatía sensitiva y autonómica como manifestaciones neurológicas paraneoplásicas. Una vez iniciado el tratamiento específico para su lin foma, la neuronopatía autonómica tuvo resolución casi completa a diferencia de la neuronopatía sensitiva, la cual demostró escasa recuperación.

Abstract Hodgkin lymphoma (HL) comprises a heterogeneous group of lymphoid neoplasms whose origin lies in B lym phocytes. The neurological manifestations of this pathol ogy are infrequent, and may arise from direct invasion of neoplastic cells to the nervous system, or indirectly, through paraneoplastic syndromes or as a complication of treatment. Among the neurological paraneoplastic syndromes that affect patients with HL, paraneoplastic cerebellar degeneration is the most common. Other few cases include limbic encephalitis, sensory, motor, and autonomic neuronopathy. These syndromes can be the initial manifestation of neoplastic disease, and the lack of information regarding this association can lead to a delay in diagnosis and consequently in the initiation of therapy worsening the prognosis. We report the case of a woman with HL who presented sensory and autonomic neuronopathy at the onset of her disease as paraneo plastic neurological manifestations. After the initiation of the specific treatment for the lymphoma, the autonomic neuronopathy had almost complete resolution, unlike the sensory neuronopathy, which showed limited recovery.

[Hodgkin's lymphoma: sensitive and autonomic neuropathy as a paraneoplastic manifestation]. / Linfoma de Hodgkin: neuronopatía sensitiva y autonómica como manifestación paraneoplásica.

Hodgkin lymphoma (HL) comprises a heterogeneous group of lymphoid neoplasms whose origin lies in B lymphocytes. The neurological manifestations of this pathology are infrequent, and may arise from direct invasion of neoplastic cells to the nervous system, or indirectly, through paraneoplastic syndromes or as a complication of treatment. Among the neurological paraneoplastic syndromes that affect patients with HL, paraneoplastic cerebellar degeneration is the most common. Other few cases include limbic encephalitis, sensory, motor, and autonomic neuronopathy. These syndromes can be the initial manifestation of neoplastic disease, and the lack of information regarding this association can lead to a delay in diagnosis and consequently in the initiation of therapy worsening the prognosis. We report the case of awoman with HL who presented sensory and autonomic neuronopathy at the onset of her disease as paraneoplastic neurological manifestations. After the initiation of the specific treatment for the lymphoma, the autonomic neuronopathy had almost complete resolution, unlike the sensory neuronopathy, which showed limited recovery.

El linfoma de Hodgkin (LH) comprende un grupo heterogéneo de neoplasias linfoides cuyo origen radica en linfocitos B. Las manifestaciones neurológicas de dicha enfermedad son infrecuentes, pudiendo tener su origen por invasión directa de las células neoplásicas en el sistema nervioso, o indirectamente, a través de síndromes paraneoplásicos o como complicación del tratamiento. Dentro de los síndromes neurológicos paraneoplásicos que afectan a pacientes con LH, la degeneración cerebelosa paraneoplásica es la más frecuente. Otros reportados con menor frecuencia en series de casos o casos aislados incluyen encefalitis límbica, neuronopatía sensitiva, motora y autonómica. Estos pueden ser la manifestación inicial de la enfermedad neoplásica, y la falta de conocimiento de dicha asociación puede retrasar el diagnóstico, con inicio tardío del tratamiento y peor pronóstico. Reportamos el caso de una mujer con LH que presentó al inicio de su enfermedad neuronopatía sensitiva y autonómica como manifestaciones neurológicas paraneoplásicas. Una vez iniciado el tratamiento específico para su linfoma, la neuronopatía autonómica tuvo resolución casi completa a diferencia de la neuronopatía sensitiva, la cual demostró escasa recuperación.

Clinical significance of V617F mutation of the JAK2 gene in patients with chronic myeloproliferative disorders.

OBJECTIVE: To determine the prevalence of JAK2 V617F mutation and its clinical correlation in patients with chronic myeloproliferative disorders (CMD): polycythemia vera (PV), essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF). MATERIALS AND METHODS: Detection of JAK2 V617F mutation by allele specific-PCR. RESULTS: One hundred and three patients with CMD were included in the study. JAK2 V617F distribution was PV 40/45 (89%), ET 30/43 (69%), and IMF 7/15 (47%). In PV and ET patients only, 18 had thrombosis at diagnosis and 12 during follow-up (these were microvascular: 11, venous: 7 and arterial: 12); of these 28/70 (40%) were JAK2pos versus 2/18 (11%) JAK2neg; P=0.02. In a median of 4 years, two patients with PV JAK2pos evolved to myelofibrosis and one patient with PV presented in leukemic transformation (JAK2pos before and after transformation); six patients died: four patients with IMF and two patients with PV. CONCLUSIONS: We found an association between JAK2 V617F and thrombotic events in patients with PV and ET.

Clinical outcome of patients with relapsed/refractory acute leukemia treated with FLAG-IDA regimen

Background: Patients with Refractory/Relapsed (RIR) acute leukemia (AL) have a poor prognosis. Objective: We aimed to evaluate the chemotherapy regimen fludarabine, cytarabine, granulocyte colony-stimulating factor, and idarubicin (FLAG-IDA) in patients with RIR AL. Patients: We studied 33 patients with R/R AL. Distribution of the AL subtype was: myeloblastic n=17 (52%), lymphoblastic n=14 (42%),) and biphenotypic n=2 (6%). Results: Complete remission (CR) was achieved in 15 cases (45.5%) and seven patients dead resulting in a mortality of 21.1%. In patients with hematological recovery the median time to neutrophils recovery (> 0.5 x 10º/1) was 24 days (range 10-38); platelet levels of more than 20 x 1Oº/l and 50 x 10º/1 were reached in a median time of 24 (range 17-44) and 27 days (range 18-51), respectively. After CR, five patients underwent allogeneic transplan- tation and 10 patients received a second course of FLAG – IDA. Ten out of 15 patients who achieved CR with FLAG-IDA relapsed at a median of 7.7 months (95% CI 1.8 to 13.6 months). Overall survival (OS) after FLAG-IDA in the surviving cohort had a median of 4 months. We found a significantly better OS in patients who received allogeneic transplantation post-FLAG-IDA than those who did not (median 11.4 months vs. 2.7 monthsj HR 0.29; 95% CI 0.1 to 0.6; p=0.017). Conclusions: In our series, FLAG-IDA demonstrated to be an effective salvage chemotherapy regimen, however, the benefit in survival of this rescue treatment was restrained to patients who unde.rwent al1ogeneic transplantation