Hematological Neoplasms with Eosinophilia.

Eosinophils in peripheral blood account for 0.3-5% of leukocytes, which is equivalent to 0.05-0.5 × 109/L. A count above 0.5 × 109/L is considered to indicate eosinophilia, while a count equal to or above 1.5 × 109/L is defined as hypereosinophilia. In bone marrow aspirate, eosinophilia is considered when eosinophils make up more than 6% of the total nuclear cells. In daily clinical practice, the most common causes of reactive eosinophilia are non-hematologic, whether they are non-neoplastic (allergic diseases, drugs, infections, or immunological diseases) or neoplastic (solid tumors). Eosinophilia that is associated with a hematological malignancy may be reactive or secondary to the production of eosinophilopoietic cytokines, and this is mainly seen in lymphoid neoplasms (Hodgkin lymphoma, mature T-cell neoplasms, lymphocytic variant of hypereosinophilic syndrome, and B-acute lymphoblastic leukemia/lymphoma). Eosinophilia that is associated with a hematological malignancy may also be neoplastic or primary, derived from the malignant clone, usually in myeloid neoplasms or with its origin in stem cells (myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions, acute myeloid leukemia with core binding factor translocations, mastocytosis, myeloproliferative neoplasms, myelodysplastic/myeloproliferative neoplasms, and myelodysplastic neoplasms). There are no concrete data in standardized cytological and cytometric procedures that could predict whether eosinophilia is reactive or clonal. The verification is usually indirect, based on the categorization of the accompanying hematologic malignancy. This review focuses on the broad differential diagnosis of hematological malignancies with eosinophilia.

Peripheral T-cell lymphoma with a T follicular-helper phenotype: A different entity? Results of the Spanish Real-T study.

Nodal peripheral T-cell lymphoma (PTCL) with a T follicular helper phenotype (PTCL-TFH) is a new type of PTCL. We aimed to define its clinical characteristics and prognosis compared to PTCL not otherwise specified (PTCL-NOS) and angioimmunoblastic T-cell lymphoma (AITL). This retrospective observational study included 175 patients diagnosed with PTCL between 2008 and 2013 in 13 Spanish sites. Patient diagnosis was centrally reviewed, and patients were reclassified according to the World Health Organization (WHO) 2016 criteria: 21 patients as PTCL-NOS, 55 as AITL and 23 as PTCL-TFH. Median follow-up was 56.07 months (95% CI 38.7-73.4). Progression-free survival (PFS) and overall survival (OS) were significantly higher in patients with PTCL-TFH than in those with PTCL-NOS and AITL (PFS, 24.6 months vs. 4.6 and 7.8 months, respectively, p = 0.002; OS, 52.6 months vs. 10.0 and 19.3 months, respectively, p < 0.001). Histological diagnosis maintained an independent influence on both PFS (hazard ratio [HR] 4.1 vs. PTCL-NOS, p = 0.008; HR 2.6 vs. AITL, p = 0.047) and OS (HR 5.7 vs. PTCL-NOS, p = 0.004; HR 2.6 vs. AITL, p = 0.096), regardless of the International Prognostic Index. These results suggest that PTCL-TFH could have more favourable features and prognosis than the other PTCL subtypes, although larger series are needed to corroborate these findings.

Clinical and pathological characteristics of peripheral T-cell lymphomas in a Spanish population: a retrospective study.

We investigated the clinicopathological features and prognostic factors of patients with peripheral T-cell lymphoma (PTCL) in 13 sites across Spain. Relevant clinical antecedents, CD30 expression and staining pattern, prognostic indices using the International Prognostic Index and the Intergruppo Italiano Linfomi system, treatments, and clinical outcomes were examined. A sizeable proportion of 175 patients had a history of immune-related disorders (autoimmune 16%, viral infections 17%, chemo/radiotherapy-treated carcinomas 19%). The median progression-free survival (PFS) and overall survival (OS) were 7·9 and 15·8 months, respectively. Prognostic indices influenced PFS and OS, with a higher number of adverse factors resulting in shorter survival (P < 0·001). Complete response (CR) to treatment was associated with better PFS (62·6 vs. 4 months; P < 0·001) and longer OS (67·0 vs. 7·3 months; P < 0·001) compared to no CR. CD30 was expressed across all subtypes; >15% of cells were positive in anaplastic lymphoma kinase-positive and -negative anaplastic large-cell lymphoma and extranodal natural killer PTCL groups. We observed PTCL distribution across subtypes based on haematopathological re-evaluation. Poor prognosis, effect of specific prognostic indices, relevance of histopathological sub-classification, and response level to first-line treatment on outcomes were confirmed. Immune disorders amongst patients require further examination involving genetic studies and identification of associated immunosuppressive factors.

Neuropsychiatric Aspects in a Patient Diagnosed with Corticobasal Degeneration: Clinical Case of Low Incidence and Prevalence in Colombia.

Corticobasal degeneration (CBD) is a pathology of low incidence and prevalence worldwide; it is accompanied by symptoms such as dystonia, rigid akinetic syndrome (bradykinesia), gait disturbances, neurological deterioration associated with severe cortical subcortical atrophy, and progressive to moderate to severe neurocognitive deficits, especially in immediate verbal memory and dorsolateral or dysexecutive syndrome. We identified neurocognitive impairment and neuropsychiatric symptoms in a patient diagnosed with CBD. Participant was a 70-year-old female patient, single; she presented progressive memory loss of an immediate verbal nature. Initially, she was diagnosed with Alzheimer's disease and Lewy body dementia, finding that she had no characteristic signs and symptoms of these pathologies. The patient presented conciliation insomnia, gait disturbances, and severe neurocognitive deficit, especially in executive functions, immediate verbal memory, and visuospatial functioning. It was found that the patient presented neurocognitive alterations of the executive type (frontal lobe) such as decision making, planning, inhibition and operative memory, correlated with a severe alteration in her basic, instrumental and advanced activities of daily life, with a high risk factor for developing dementia. It is necessary to diagnose in an assertive and timely manner in order to generate functional neurorehabilitation plans in people diagnosed with CBD, with the main objective of positively impacting quality of life, at the individual, family, and social level.

Forma quística del tumor estromal esclerosante de ovario / Sclerosing cystic stromal tumor of the ovary

Introducción: El tumor estromal esclerosante es una neoplasia benigna que representa el 2-6% de los tumores estromales ováricos, y el 80% se presenta en mujeres entre la segunda y tercera décadas de la vida. Suelen ser tumores sólidos con áreas de edema y pequeñas zonas quísticas y un patrón histológico característico. Descripción del caso: Presentamos un caso de tumor estromal esclerosante totalmente quístico en una paciente de 53 años de edad, planteándose el diagnóstico diferencial con otras lesiones quísticas ováricas. Conclusión: El tumor esclerosante ovárico es un tumor benigno que puede plantear el diagnóstico diferencial tanto con lesiones ováricas sólidas como quísticas

Introduction: Sclerosing stromal tumor is a benign neoplasm representing 2-6% of ovarian stromal tumors. Eighty percent of these tumors appear in women in the second and third decades of life. These neoplasms are usually macroscopically solid tumors with small edematous and cystic areas and show characteristic histological features. Case report: We report a case of sclerosing cystic stromal tumor of the ovary in a 53-year-old woman and discuss its differential diagnosis with other solid and cystic ovarian neoplasms. Conclusion: Sclerosing ovarian tumor is a benign entity that may pose problems of differential diagnosis with other solid and cystic tumors

Sarcoma del estroma prostático en un paciente de 20 años de edad / Prostatic stromal sarcoma in a 20-year-old patient

OBJETIVOS: Los tumores de próstata representanla neoplasia maligna más frecuente en el varón.Mientras que las lesiones epiteliales son frecuentes, lasneoplasias de origen mesenquimal representan solo un0´2% de las neoplasias malignas prostáticas y son pococonocidas, sobre todo las de origen estromal.METODOS Y RESULTADOS: Este artículo describe un casode un varón de 20 años de edad, con un sarcoma delestroma prostático al que se le realiza una cistoprostatectomíaradical. El tumor ocupa prácticamente la totalidadde la próstata, alcanzando un diámetro de 8 cmHistológicamente está constituido por células fusiformescon moderada atipia y frecuentes figuras de mitosis, entremezcladascon aisladas estructuras ductales elongadas,adoptando un patrón similar al tumor filodes, que planteael diagnóstico diferencial con otros tumores mesenquimalesprostáticos. CONCLUSIONES: El comportamiento clínico de estostumores, basado en los escasos casos descritos en la literatura,es prácticamente desconocido . Las recurrenciaslocales no son raras, y se han descrito casos de metástasisa distancia, fundamentalmente a pulmón y hueso. Ennuestro caso el paciente permanece libre de enfermedada los 12 meses del diagnóstico

OBJECTIVE: Prostatic tumors are the most frequent malignant neoplasms in men, most of them being constituted by carcinomas; only 0,2% of malignant prostatic neoplasms are of mesenchimal origin. They are not well known, especially those of prostatic stromal phenotype. METHODS AND RESULTS: We report the case of a 20- year-old man with a prostatic stromal sarcoma. After total cystoprostatectomy a tumor measuring 8 cm could be seen, replacing almost the whole prostate. Microscopically a spindle cell neoplasia with moderate atypia and a high mitotic index entrapping few elongated prostatic ducts (adopting a phyllodes tumor morphology) was observed. CONCLUSION: The clinical behavior of these infrequent sarcomas is not well-established. Recurrences are not uncommon whereas lung and bone metastases have been described. Twelve months after surgery our patient is alive without evidence of disease

Hereditary vacuolar internal anal sphincter myopathy causing proctalgia fugax and constipation: a new case contribution.

Hereditary anal sphincter myopathy is rare. We present a family with one affected member with proctalgia fugax, constipation and internal anal sphincter hypertrophy. Ultrastructural findings show vacuolization of smooth muscle cells without the characteristic polyglucosan inclusion. Further relief of symptoms was obtained using an oral calcium antagonist. Based on clinical presentation, endosonography and morphological findings, we consider our case is a histological variant of the vacuolar myopathy originally described.

[Prostatic stromal sarcoma in a 20-year-old patient]. / Sarcoma del estroma prostático en un paciente de 20 años de edad.

OBJECTIVE: Prostatic tumors are the most frequent malignant neoplasms in men, most of them being constituted by carcinomas; only 0.2% of malignant prostatic neoplasms are of mesenchimal origin. They are not well known, especially those of prostatic stromal phenotype. METHODS AND RESULTS: We report the case of a 20 year-old man with a prostatic stromal sarcoma. After total cystoprostatectomy a tumor measuring 8 cm could be seen, replacing almost the whole prostate. Microscopically a spindle cell neoplasia with moderate atypia and a high mitotic index entrapping few elongated prostatic ducts (adopting a phyllodes tumor morphology) was observed. CONCLUSION: The clinical behavior of these infrequent sarcomas is not well-established. Recurrences are not uncommon whereas lung and bone metastases have been described. Twelve months after surgery our patient is alive without evidence of disease.