Are accessory spleen screening and resection in refractory immune cytopenia an effective strategy or a waste of resources?

Splenectomy remains an effective treatment for refractory immune cytopenia (RIC), which encompasses immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Accessory spleens (AS) have been described without identifying specific risk factors. We retrospectively analyzed patients with RIC after splenectomy who underwent splenic scintigraphy (SS) at our institution. Seventy-one patients were included. Sixty-two patients had ITP, five had AIHA, and four had Evans syndrome. Seventy-five percent (n = 53) were women. Eleven patients (15.5%) had an AS detected by SS. A complete response (CR) to first-line steroids (odds ratio (OR) 5.75, 95% confidence interval (CI) 1.37-24.14, p = 0.017) and the absence of Howell-Jolly bodies (HJB) in peripheral blood smear (PBS) (OR 11.37, 95% CI 2.70-47.85, p = 0.001) were found to be risk factors. Patients with both elements had a higher rate of AS (83.3%) when compared to those with one or no factors (p < 0.001). Eight patients (73%) underwent an accessory splenectomy: seven (87.5%) achieved a CR, and none had perioperative complications. The presence of HJB in PBS changed from 25 to 87.5% after accessory splenectomy. We recommend the search for an AS via SS in patients with RIC due to ITP, who had a CR to corticosteroids and the absence of HJB in PBS. Accessory splenectomy is a safe and effective procedure.

Bazo accesorio secundario a trombocitopenia autoinmune primaria refractaria - Un reto diagnóstico / Accessory spleen resection in refractory primary autoimmune thrombocytopenia - a diagnostic challenge

Resumen En la trombocitopenia autoinmune primaria hay formación de autoanticuerpos contra las membranas plaquetarias causando destrucción plaquetaria esplénica y compromiso clínico consistente en petequias, hemorragias y trombocitopenia. Cuando falla el manejo médico con corticoides e inmunoglobulinas está indicada la realización de esplenectomía, que funciona para remisión parcial de la enfermedad persistente. Realizar la esplenectomía hace necesario el estudio de un bazo accesorio o material esplénico recurrente, que comúnmente se asocia con la pobre respuesta a tratamiento y recaídas de la enfermedad. Se presenta el caso de una paciente de 27 años y antecedentes de trombocitopenia autoinmune primaria quien requirió esplenectomía, con adecuada evolución postoperatoria y remisión de los síntomas por dos años. Posteriormente, presentó recaída clínica y paraclínica, con plaquetas en 11 000 cel/uL, en contexto de trombocitopenia severa sintomática, presencia de gingivorragia, petequias en los miembros inferiores y el abdomen. Se realizó búsqueda activa de bazo supernumerario por medio de estudios complementarios, los cuales evidenciaron bazo accesorio localizado en el ligamento gastro cólico, que fue resecado por vía laparoscópica, con adecuada respuesta y remisión sintomática de la trombocitopenia autoinmune primaria refractaria. En pacientes esplenectomizados con púrpura trombocitopénica idiopática que tengan recaída de su enfermedad se requiere búsqueda activa de bazos supernumerarios, puesto que es una causa frecuente de su reaparición.

Abstract In primary autoimmune thrombocytopenia there is formation of autoantibodies against the splenic platelet membranes causing platelet destruction with clinical deterioration consisting of petechiae, haemorrhages and thrombocytopenia. When the medical management with corticoids and immunoglobulins fails, it is indicated the accomplishment of splenectomy that works for partial remission of the persistent disease. Performing splenectomy requires the study of an accessory spleen or the recurrent splenic material, that is commonly associated with poor response to treatment and frequent relapses of primary autoimmune thrombocytopenia. A case is presented in a 27-year-old patient with a history of primary autoimmune thrombocytopenia requiring splenectomy, with adequate postoperative evolution and remission of symptoms for two years who later presents clinical and paraclinical relapse with platelets at 11,000 cells/uL in the context of severe symptomatic thrombocytopenia, with the presence of gingivorrhagia, petechiae in the lower limbs and abdomen. Active search of supernumerary spleen was performed by means of complementary studies that showed an accessory spleen located in the gastrocolic ligament, which was resected by laparoscopy with adequate symptomatic remission response of the refractory primary autoimmune thrombocytopenia. In splenectomized patients with idiopathic thrombocytopenic purpura who have relapsed their disease, they require an active search for supernumerary spleens since it is a frequent cause of their reappearance.

Presencia y biometría de bazo accesorio en individuos chilenos: estudio por tomografía computarizada / Presence and biometry of accessory spleen in Chilean individuals: study by computerized tomography

El bazo se localiza en el cuadrante superior izquierdo del abdomen, relacionándose posteriormente con la 9a a 11a costilla, de las que se separa por el diafragma y el receso costodiafragmático, se localiza por detrás del estómago y lateralmente al riñón izquierdo. Por alteraciones en su desarrollo pueden generarse bazos accesorios (BA), considerándose un tejido ectópico del bazo. Se consideran tejido normal, con los mismos procesos fisiológicos que el bazo principal. Con el propósito de localizar y determinar aspectos biométricos de los mismos, se realizó un estudio de corte transversal y de carácter descriptivo, sobre una muestra de 220 exámenes de TC pertenecientes a pacientes mayores de 18 años del Hospital Regional Hernán Henríquez Aravena, Temuco, Chile. Para este estudio se excluyeron toda aquellas TC con antecedentes de esplenectomía y lesiones de Bazo o peri-esplénicas. El análisis de los datos mostró una prevalencia de 32,3 % de BA, pudiendo ser de una única presencia, dos e incluso tres BA por paciente.De un total de 71 personas que tienen al menos un BA, 34 (47,89 %) fueron de sexo femenino y 37 (52,11 %) de sexo masculino. Hubo 56 pacientes (78,9 %) con un BA, 29 (40,85 %) del sexo femenino y 27 (38,03 %) del masculino; 15 (21,1 %) presentaron más de un BA, 5 (7,04 %) de sexo femenino y 10 (14,08 %) de sexo masculino, si bien se puede observar variación en la cantidad de BA según sexo, no existe una relación estadísticamente significativa entre dichas variables. La ubicación más frecuente encontrada en el plano axial fue la zona antero-medial con 59 casos (66,29 %); asimismo, en el plano sagital, la localización más frecuente fue en el polo inferior con 40 casos (44,44 %). Datos biométricos de estos BA son mostrados en Tablas. Esta información será de gran valor morfológico y médico debido a la escasa literatura existente sobre esta materia en individuos chilenos.

The spleen is located in the upper left quadrant of the abdomen, subsequently related to the 9th to 11th rib, from which it is separated by the diaphragm and the cost-diaphragmatic recess, it is located behind the stomach and laterally to the left kidney. Due to alterations in its development, accessory spleens (AS) can be generated, being considered an ectopic tissue of the spleen. The AS are considered normal tissue, with the same physiological processes as the main spleen. With the purpose of locating and determining biometric aspects of them, a cross-sectional and descriptive study was carried out on a sample of 220 CT scans belonging to patients over 18 years of age at the Hernán Henríquez Aravena Regional Hospital, Temuco, Chile. For this study, all CT scans with a history of splenectomy and spleen or peri-splenic lesions were excluded. The analysis of the data showed a prevalence of 32.3 % of AS, being able to be of a single presence, two and even three AS per patient. Of a total of 71 people who have at least one AS, 34 (47.89 %) were female and 37 (52.11 %) male. There were 56 patients (78.9 %) with a one AS, 29 (40.85 %) of the female sex and 27 (38.03 %) of the male; 15 (21.1 %) presented more than one AS, 5 (7.04 %) female and 10 (14.08 %) male, although variation in the amount of AS according to sex can be observed, no there is a statistically significant relationship between these variables. The most frequent location found in the axial plane was the anteromedial zone with 59 cases (66.29 %); also, in the sagittal plane, the most frequent location was in the lower pole with 40 cases (44.44 %). Biometric data of these AS are shown in tables. This information will be of great morphological and medical value due to the limited existing literature on this subject in Chilean individuals.

Torção esplênica primária associado à baço acessório em cão / Primary splenic torsion associated with accessory spleen in a dog

Background: Primary splenic torsion occurs when there is a splenic vascular pedicle rotation without association of otherdiseases, as the Gastric Dilatation Volvulus (GDV). Accessory spleen consists in a splenic tissue fraction independentfrom main organ, caused by a spleen defect in the embryonic development, most remaining asymptomatic and discoveredincidentally. They have their own blood supply, which is usually from a branch of the splenic artery. Present report represents a primary splenic torsion with the accessory spleen finding in a dog.Case: A 7-year-old female mongrel was referred to Veterinary Hospital of Universidade Federal do Rio Grande do Sul,showing pain and firm consistency in the left abdominal region, prostration and inappetence four days before the medicalappointment. At the clinical examination, the patient was alert, 7% dehydration, hypocorous mucous membranes, withoutalteration in lymph nodes, left abdominal pain. Laboratory tests, chest radiographic and abdominal ultrasound was requested.Two images compatible with splenic image were visualized, one with a regular structure without vascularization suggestingsplenic torsion and another with homogeneous parenchyma and echogenicity of the normal spleen, with vascularizationsignal. The animal was referred to the surgical procedure and the anesthetic induction protocol with propofol (6 mg/kg-1)and ketamine hydrochloride (2 mg/kg-1) and maintenance with total intravenous anesthesia with propofol (0.2-0.4 mg/kg-1/min) and lidocaine (2 mg/kg-1), in a semi-closed loop and spontaneous ventilation. The pre-retro-umbilical incision in theventral midline was performed, and the exploratory celiotomy was done. During the cavity exploring, a small amount of freefluid and a structure compatible with the spleen were observed. It was noted that the splenic vascular pedicle was twistedaround its axis and the gastroesplenic and splenocolic ligaments were also involved...(AU)

Torção esplênica primária associado à baço acessório em cão / Primary splenic torsion associated with accessory spleen in a dog

Background: Primary splenic torsion occurs when there is a splenic vascular pedicle rotation without association of otherdiseases, as the Gastric Dilatation Volvulus (GDV). Accessory spleen consists in a splenic tissue fraction independentfrom main organ, caused by a spleen defect in the embryonic development, most remaining asymptomatic and discoveredincidentally. They have their own blood supply, which is usually from a branch of the splenic artery. Present report represents a primary splenic torsion with the accessory spleen finding in a dog.Case: A 7-year-old female mongrel was referred to Veterinary Hospital of Universidade Federal do Rio Grande do Sul,showing pain and firm consistency in the left abdominal region, prostration and inappetence four days before the medicalappointment. At the clinical examination, the patient was alert, 7% dehydration, hypocorous mucous membranes, withoutalteration in lymph nodes, left abdominal pain. Laboratory tests, chest radiographic and abdominal ultrasound was requested.Two images compatible with splenic image were visualized, one with a regular structure without vascularization suggestingsplenic torsion and another with homogeneous parenchyma and echogenicity of the normal spleen, with vascularizationsignal. The animal was referred to the surgical procedure and the anesthetic induction protocol with propofol (6 mg/kg-1)and ketamine hydrochloride (2 mg/kg-1) and maintenance with total intravenous anesthesia with propofol (0.2-0.4 mg/kg-1/min) and lidocaine (2 mg/kg-1), in a semi-closed loop and spontaneous ventilation. The pre-retro-umbilical incision in theventral midline was performed, and the exploratory celiotomy was done. During the cavity exploring, a small amount of freefluid and a structure compatible with the spleen were observed. It was noted that the splenic vascular pedicle was twistedaround its axis and the gastroesplenic and splenocolic ligaments were also involved...

Macroscopic and microscopic findings in a set of congenital anomalies in two calves produced through in vitro production

In vitro production and somatic cell nuclear transfer are biotechnologies widely used for breeding cattle, although may result in congenital anomalies. This paper aims to report a set of congenital anomalies in two Nelore calves, a male and a female, produced through in vitro fertilization. The major anomalies revealed at necropsy were hypospadias, bifid scrotum, atresia ani and rectum ending in blind pouch in the male calf. In the female calf accessory spleen, atresia ani, underdevelopment of extern genitalia and urethral orifice, and rectum ending in blind pouch forming a uterus-rectum fistula were observed.(AU)

Macroscopic and microscopic findings in a set of congenital anomalies in two calves produced through in vitro production

In vitro production and somatic cell nuclear transfer are biotechnologies widely used for breeding cattle, although may result in congenital anomalies. This paper aims to report a set of congenital anomalies in two Nelore calves, a male and a female, produced through in vitro fertilization. The major anomalies revealed at necropsy were hypospadias, bifid scrotum, atresia ani and rectum ending in blind pouch in the male calf. In the female calf accessory spleen, atresia ani, underdevelopment of extern genitalia and urethral orifice, and rectum ending in blind pouch forming a uterus-rectum fistula were observed.

Baço acessório intrapancreático: achados de imagem / Intrapancreatic accessory spleen: imaging findings

Baços acessórios consistem em proliferações de tecido esplênico, com prevalência relatada de 10% a 30% em estudos de autópsias e de 45% a 65% em pacientes submetidos a esplenectomia. Quando este tecido se localiza em meio ao tecido pancreático, são denominados baços acessóriosintrapancreáticos, sendo esta a segunda localização mais frequente, representando cerca de 16,8%dos casos. Esta entidade apresenta um interesse particular, por tratar-se de l esão benigna, geralmente incidental, cujo diagnóstico diferencial deve ser feito com lesões primárias pancreáticas.Neste caso devem ser afastados tumores neuroendócrinos (de ilhotas pancreáticas), adenocarcinoma e metástases. Na investigação por imagem, os baços acessórios intrapancreáticos se manifestamcom aspecto semelhante ao baço nativo em todos os métodos. À ultrassonografia, aparecem como imagem circunscrita hipoecóica homogênea, e na tomografia computadorizada e ressonânciamagnética apresentam, respectivamente, densidade, características de sinal e realce semelhantes ao baço. Nos casos em que o aspecto de imagem permanecer inespecífico, o diagnósticopode ser sugerido pela cintilografia com hemácias marcadas com tecnécio-99m, atualmente considerado o exame padrão ouro. Todas estas modalidades fornecem elementos para o diagnósticocom segurança, evitando procedimentos invasivos desnecessários.

Accessory spleens consist of spleen tissue proliferations, with an estimated prevalence of 10% to 30% in autopsies studies and 45% to 65% in patients submitted to splenectomy. Accessory spleens can occur within the pancreatic tissue (intrapancreatic accessoryspleen), representing the second most frequent localization (about 16.8% of the cases). This entity is of particular interest, since it is a benign lesion, generally incidentally detected, that should be differentiated from pancreatic endocrine neoplasm, pancreatic adenocarcinoma and metastasis. Intrapancreatic accessory spleen has similar imaging manifestations to native spleen in all imaging modalities. At ultrasound, it appears as a homogeneous, hypoechoic, circumscribed image. At computed tomography and magnetic resonance imaging it has similar density, signal characteristics and enhancement pattern in comparison to the spleen. In somecases, where the image findings remain nonspecific, the diagnosis may be suggested by technetium-99m radiolabeled red blood cells scintigraphy, currently considered the gold standard test. This aspect allows a confident imaging diagnosis, preventing unnecessary invasive procedures.