Paraspinal extramedullary hematopoiesis in hereditary spherocytosis with a concurrent follicular lymphoma: case report and review of the literature.

We report an unusual case of a 70-year-old male with history of hereditary spherocytosis (HS) and secondary paraspinal extramedullary hematopoiesis with a concurrent follicular lymphoma. The lesion presented as a thoracic paraspinal mass of 9 cm, extending longitudinally between T6 and T9 vertebral bodies. Incisional biopsy revealed that this mass included mature hematopoietic tissue compatible with extramedullary hematopoiesis (EMH). The tissue also presented an extensive and diffuse infiltration by an atypical lymphoid population composed predominantly by small cells. The immunohistochemical study revealed that the atypical lymphoid population had a germinal center phenotype, consistent with the diffuse variant of follicular lymphoma (FL). The simultaneous presence of both EMH and FL in the same lesion made the interpretation and the final diagnosis of this case difficult. The presence of EMH in this clinical context may eclipse the diagnosis of the underlying lymphoproliferative neoplasm. The close association between the tumor cells and extramedullary hematopoietic tissue in the absence of lymphadenopathies or other tissue involvement suggests a relationship of this tumor with the recently described primary FL of the bone marrow.

[Hereditary spherocytosis. Review. Part II. Symptomatology, outcome, complications, and treatment]. / Esferocitosis hereditaria. Revisión. Parte II. Manifestaciones clínicas, evolución, complicaciones y tratamiento.

Hereditary spherocytosis must always be suspected in children with anemia, hyperbilirubinemia, splenomegaly or cholelithiasis, in the asymptomatic individual with an affected relative, and in the neonate with hyperbilirubinemia with no blood group incompatibility; its early detection is key to avoid kernicterus. Follow-up of these patients is based on periodical control and supply of information on the adequate management of hemolytic or aplastic crisis, and early detection of cholelithiasis. The decision to perform splenectomy is usually associated with quality of life rather than life-threatening risk, and it should result from a consensus between patient, parents and physicians. The postsplenectomy follow-up is based on control of compliance with the prophylactic antibiotic therapy and the early diagnosis of infectious disorders.

[Anaemia in adopted children, not always iron deficiency]. / Anemie bij adoptiekinderen, niet altijd ijzergebrek.

Anaemia was diagnosed in four adopted children during a standard screening examination 1-4 weeks after arrival. Further investigation revealed a number of causes which could then be specifically treated. The children were a girl aged 14 months from China with iron-deficiency anaemia, a boy aged 16 months from Nigeria with sickle cell anaemia, a girl aged 5 from Haiti who had alpha-thalassaemia, and a boy aged 7 from Brazil with spherocytosis. Iron deficiency is the most common cause of anaemia in childhood. However, in adopted children from sub-tropical areas other causes of anaemia like haemoglobinopathies or erythrocyte membrane defects should be borne in mind, particularly as a history of disease and family history are often lacking. Additional investigations may be necessary. An incorrect diagnosis of iron deficiency may result in ongoing and unjustified iron supplementation leading to harmful iron accumulation in thalassaemia and a delay in the correct treatment in sickle cell anemia or spherocytosis which could carry considerable risk.

Leg ulcer in hereditary spherocytosis.

Indolent leg ulcers are a rare complication found in patients with hereditary spherocytosis. We report a 13-year-old girl with hereditary spherocytosis who developed a chronic painful ulcer on the medial malleolus. All other etiologies were ruled out. Nine months after splenectomy the ulcer healed completely and the symptoms disappeared. We discuss and review this unusual entity in children.

[Hemochromatosis associated with hereditary spherocytosis]. / Asociación de hemocromatosis y esferocitosis hereditaria.

Hereditary spherocytosis is a chronic hemolytic anemia that very infrequently produces severe iron overload. Only 15 cases of hereditary spherocytosis associated with hemochromatosis have been described previously. It was initially thought that hemochromatosis was the result of the increase of iron stores secondary to chronic hemolysis. Afterwards, it became apparent that iron overload could appear in patients splenectomized. This fact suggested that spherocytosis and idiopathic hemochromatosis could be inherited independently. We describe the case of a 45-year-old man, with known hereditary spherocytosis, splenectomized at 5 years of age, who developed iron overload which affected his heart, liver and pancreas.

[Video laparoscopic splenectomy in a patient with splenomegaly due to hereditary spherocytosis: report of two cases]. / Esplenectomia videolaparoscópica em paciente portador de esplenomegalia por esferocitose hereditária: relato de dois casos.

We present a case report of laparoscopic splenectomy in two patients with congenital spherocytosis. A 23-year-old man and a 25-year-old woman with splenomegaly due to congenital spherocytosis underwent laparoscopic splenectomy. Their postoperative course was uncomplicated and they were discharged on the second and fourth postoperative day, respectively.

Posibilidades actuales de la cirugía laparoscópica: colecistectomia y esplenectomía simultáneas / Reaching of laparoscopic surgery: cholocystectomy and splenectomy in same operation

Se hace una breve reseña sobre los alcances de la cirugía laparoscópica y se comenta la observación de una paciente portadora de una esferocitosis a la cual se le practicó una colecistectomía y esplenectomía simultáneas con ese procedimiento y que presentó una seria complicación postoperatoria por la que debió ser reoperada pero que finalmente evolucionó bien

Posibilidades actuales de la cirugía laparoscópica: colecistectomia y esplenectomía simultáneas / Reaching of laparoscopic surgery: cholocystectomy and splenectomy in same operation

Se hace una breve reseña sobre los alcances de la cirugía laparoscópica y se comenta la observación de una paciente portadora de una esferocitosis a la cual se le practicó una colecistectomía y esplenectomía simultáneas con ese procedimiento y que presentó una seria complicación postoperatoria por la que debió ser reoperada pero que finalmente evolucionó bien

Aplastic crisis due to human parvovirus B19 infection in hereditary hemolytic anaemia

Specific anti-B19 IgM was demonstrated in sera from three children showing transient aplastic crisis. A two years-old boy living in Rio de Janeiro suffering from sickle-cell anaemia showed the crisis during August, 1990. Two siblings living in Santa Maria, RS, developed aplastic crisis during May, 1991, when they were also diagnosed for hereditary spherocytosis. For a third child from this same family, who first developed aplastic crisis no IgM anti-B19 was detected in her sera

Aplastic crisis due to human parvovirus B19 infection in hereditary hemolytic anaemia.

Specific anti-B19 IgM was demonstrated in sera from three children showing transient aplastic crisis. A two years-old boy living in Rio de Janeiro suffering from sickle-cell anaemia showed the crisis during August, 1990. Two siblings living in Santa Maria, RS, developed aplastic crisis during May, 1991, when they were also diagnosed for hereditary spherocytosis. For a third child from this same family, who first developed aplastic crisis no IgM anti-B19 was detected in her sera.

Recidiva em anemia esferocitica pos-esplenectomia consequente a presenca de bacos acessorios / Recurrence of spherocytic anemia postsplenectomy due to accessory spleens

Tres anos apos esplenectomia total indicada para tratamento de uma paciente portadora de anemia esferocitica se instalou quadro compativel com recidiva intensa de anemia. A investigacao clinica revelou presenca de dois bacos acessorios com 3,5 e 4 cm de diametro localizados ao ultra-som e ao mapeamento com tecnecio. Com estes achados indicou-se laparotomia que confirmou presenca dos bacos acessorios e a remocao destes normalizou quadro hematologico. O desenvolvimento esplenico, a partir da quinta semana de vida intra-uterina ao nivel do mesogastrio dorsal, com a formacao do baco embrionario multilobulado justifica a possibilidade da presenca de bacos acessorios, se nao ocorrer processo de coalescencia total dos lobulos esplenicos primitivos. Estes fatos alertam e orientam a conduta intra-operatoria que o cirurgiao deve buscar quando da realizacao de esplenectomia para o tratamento da doenca hemolitica, se recidivas decorrentes do funcionamento de bacos acessorios queiram ser evitadas.

[Extreme hyperbilirubinemia associated with spherocytosis and choledocholithiasis]. / Hiperbilirrubinemia extrema asociada a esferocitosis y coledocolitiasis.

Spherocytosis conditions the severe destruction of red blood cells. The spleen plays an active and fundamental role in this destruction since it is responsible for the fragility and lability of the red blood cell to the splenic medium. The clinical manifestations are: anemia, jaundice, splenomegaly, and hemolytic and aplastic crisis associated to viral infections. Choledocholithiasis is a manifestation of the disease which can be seen at an early stage, even in less severe cases. Choledocholithiasis is rare and this may be due to immediate surgical intervention. The levels of indirect bilirubin may vary but usually do not exceed 10 mg%, on the rising of direct bilirubin may cause suspicion of liver obstruction or damage. A case of a ten year old boy is reported who was found to have spherocytosis, with severe manifestations, who later developed cholelithiasis and total obstruction of the choledocho and in who the most important clinical manifestation was jaundice due to the increase of total bilirubin to 89 mg%, doubly checked. Both a splenectomy and a cholecystectomy were performed. Subsequent checkups were normal. These levels of bilirubin have not been previously reported. Hyperbilirubinemia; spherocytosis; choledocholithiasis.

Esferocitose com lesöes cutâneas / Spherocytosis with cutaneous lesions

Mulher com extensas lesöes cutâneas do tipo ulceroso com 10 anos de evoluçäo, alternando-se pioras e melhoras às vezes espontâneas, às vezes como resposta parcial e temporária à medicaçäo. No 10§ ano de evoluçäo chegou-se ao diagnóstico de esferocitose, procedendo-se à esplenectomia que se seguiu de melhora transitória das ulceraçöes cutâneas e do hemograma. A doente voltou a piorar e o quadro clínico prosseguiu seu curso durante mais de dois anos, findos os quais sobreveio a morte. Descreve-se pormenorizadamente a observaçäo clínica e revê-se a literatura, sem que se tenha podido explicar a patogenia das lesöes cutâneas

[Gilbert's syndrome in patients with splenomegaly]. / Síndrome de Gilbert em pacientes com esplenomegalia.

Three patients are reported with hyperbilirubinemia and splenomegaly. The diagnosis of Gilbert's syndrome was made by the caloric restriction test and enzymatic induction test. The splenomegaly was attributed to spherocytosis in two patients, while the third case had a probable diagnosis of spherocytosis.