Síndrome de Goldbloom: síndrome febril prolongado con disproteinemia. Caso clínico / Goldboom's syndrome: prolonged febrile syndrome with dysproteinemia. Case report

El síndrome de Goldbloom es una rara entidad clínica de etiología desconocida que ocurre casi exclusivamente en pediatría. Consiste en un síndrome febril prolongado con hiperostosis perióstica y disproteinemia, que, con frecuencia, simula una patología hematooncológica o linfoproliferativa. El diagnóstico se hace por exclusión de las diferentes causas de dolor de los huesos y se asocia a hipergammaglobulinemia, hipoalbuminemia, eritrosedimentación acelerada e imágenes radiológicas de periostitis. La sintomatología, la radiología y los parámetros de laboratorio remiten en un tiempo variable, que va, habitualmente, de los 3 a los 12 meses. Se presenta a un paciente de 6 años con dolores óseos difusos, hiperostosis perióstica, síndrome febril prolongado de 8 meses de evolución, pérdida de peso y reactantes de fase aguda elevados con disproteinemia (hipergammaglobulinemia e hipoalbuminemia). Debe considerarse el síndrome de Goldbloom en un paciente con las manifestaciones descritas luego de la exclusión de la patología infecciosa, hematooncológica e inflamatoria de otra causa.

Goldbloom syndrome is a rare clinical entity, of unknown etiology that happens almost exclusively in pediatric population. It is a prolonged febrile syndrome with periosteal hyperostosis and dysproteinemia, and often simulates an hematooncology or lymphoproliferative disease. The diagnosis is to rule out the different causes of bone pain associated with hypergammaglobulinemia, hypoalbuminemia, high erythrocyte sedimentation rate and periostitis at the radiographies. Symptomatology, radiology and laboratory parameters refer in a variable time, usually from 3 to 12 months. We report the case of a six-year-old boy with diffuse bone pain, prolonged febrile syndrome (of 8 months of evolution), weight loss and elevated acute phase reactants with dysproteinemia (hypergammaglobulinemia and hypoalbuminemia). Goldbloom syndrome should be considered in patients with prolonged febrile syndrome and cortical hyperostosis after the exclusion of infectious, lymphoproliferative or inflammatory disease.

On malnutrition in childhood in the Philippines: IV. Malnutrition and iron deficiency anemia

Sixty-nine cases of malnutrition in 1956, admitted to the Department of Pediatrics of the Philippine General Hospital, have been examined for iron-deficiency anemia. There is, however, no relation between the anemia, malnutrition, impairment of increase in weight and growth, and dysproteinemia in the observed cases. The frequency of iron deficiency anemia is due to the fact that diets poor in protein are, likewise, poor in iron. However, protein deficiency may also play an important role in the development or iron deficiency anemia. Therefore, the newest progress in the knowledge of protein malnutrition, the imbalance of amino acids in certain proteins and the importance of canning of food, especially milk, are discussed as factors contributing to iron deficiency anemia. Iron deficiency, if present in malnutrition, aggravates the prognosis of malnutrition considerably. (Summary and Conclusion)

A Case of Developed Tuberculous Lymphadenitis and Epstein-Barr Virus Associated Lymphadenitis / 감염

Lymphadenitis is a common pediatric disease associated with infection and inflammation. Acute lymphadenitis in children is usually accompanied by systemic viral illness, and subsides within a few days to two weeks. However, chronic lymphadenopathy or lymphadenitis is a rare disease, and is due to a reactive response to infections including tuberculosis, neoplasms, and noninflammatory lesions. The diagnostic evaluation of chronic lymphadenitis may be very complicated and it is important to consider chronic infectious diseases and rare malignancies as possible etiologies. We report a 13-year old boy who was eventually diagnosed with tuberculous lymphadenitis. The patient was initially admitted for enlarged cervical, axillary, and epitrochlear lymph nodes. Biopsy of a cervical lymph node showed the findings compatible with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) associated with EBV infection. During treatment with prednisolone, the enlarged lymph node decreased in size. However, the lymph node enlargement resumed and we performed a second biopsy. At this time the biopsy showed tuberculous lymphadenitis and a culture of gastric juice showed M. tuberculosis. With the initiation of antituberculous treatment, the patient improved clinically.

A Case of Developed Tuberculous Lymphadenitis and Epstein-Barr Virus Associated Lymphadenitis / 감염

Lymphadenitis is a common pediatric disease associated with infection and inflammation. Acute lymphadenitis in children is usually accompanied by systemic viral illness, and subsides within a few days to two weeks. However, chronic lymphadenopathy or lymphadenitis is a rare disease, and is due to a reactive response to infections including tuberculosis, neoplasms, and noninflammatory lesions. The diagnostic evaluation of chronic lymphadenitis may be very complicated and it is important to consider chronic infectious diseases and rare malignancies as possible etiologies. We report a 13-year old boy who was eventually diagnosed with tuberculous lymphadenitis. The patient was initially admitted for enlarged cervical, axillary, and epitrochlear lymph nodes. Biopsy of a cervical lymph node showed the findings compatible with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) associated with EBV infection. During treatment with prednisolone, the enlarged lymph node decreased in size. However, the lymph node enlargement resumed and we performed a second biopsy. At this time the biopsy showed tuberculous lymphadenitis and a culture of gastric juice showed M. tuberculosis. With the initiation of antituberculous treatment, the patient improved clinically.

A Case of Angioimmunoblastic Lymphadenopathy with Dysproteinemia (AILD) Like Peripheral T Cell Lymphoma / 대한소아혈액종양학회지

Angioimmunoblastic lymphadenopathy with dysproteinemia(AILD), first described at early seventies, is a clinico-pathologic syndrome which has common features of lymphadenopathy, hepatosplenomegaly, fever, rash and dysproteinemia. It is a lymphoproliferative disease, differs from malignant lymphoma by the high frequency of constitutional symptoms and by the presence of generalized lymph node enlargement, hepatosplenomegaly, cutaneous menifestations and polyclonal hypergammaglobulinemia at the time of clinical onset. Peripheral T cell lymphomas(PTCL) are morphologically and immunologically heterogeneous group of lymphoproliferative disorders that are composed of postthymic lymphocytes. There are group of cases showing features similar to AILD, and these cases are classified as AILD like PTCL. We report a rare case of AILD like PTCL in an 8 year old boy, who visited Seoul National University Hospital with fever, hepatosplenomegaly and cervical lymphadenopathy, and diagnosed as AILD like PTCL by lymph node biopsy. He did not respond to conventional chemotherapy and died 1 year after clinical onset during chemotherapy, showing feature of multiple brain infarction of unknown etiology.

A Case of Angioimmunoblastic Lymphadenopathy with Dysproteinemia with Skin Nodules / 대한피부과학회지

We report here a case of angioimmunoblastic lyinphadenopathy with dysprote inemia(AILD) in a 65-year-old male associated with skin nodules on the face. Physical examina.tion showed generalized lymphadenopathy, hepatosplenomegaly, erythematous maculopapular eruptions on the trunk and extremities, and er the matous nodules on the face. Lahoratory findings showed hemolytic anernia, arid polyclonal gammopathy. Histopathological findings of lymph node revealed typical features of AILD. I-iistopathological findings of maculopapules revealed lymphohistiocytic infiltra tion and extravasated erythrocytes around the blocked vessels, whereas histological picture of nodules on the face mirrorred that of the lymph node. After taking systemic chemotherapy, his general condition and skin lesions were improved.

A Case of angioimmunoblastic Lymphadenopathy with Dysproteinemia / 대한피부과학회지

We present a case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in a 57-year-old male who had suffered from fever and general weakness of one month's duration. He showed generalized lymphadenopathy, hepatomegaly and purpuric maculopapules on the whole body surface. Lahoratory findings show anemia, polyclonal gammaglobulinemia and thrombocytopenia. The lymph node biopsy specimens show morphologic triad of AILD except lack of interstitial deposits of eosinophilic materials and the skin biopsy specimen shows lymphohistiocytic vasculitis. Bone marrow biopsy specimen shows similar findings in lymph node, suggesting bone marrow involvement. He have been treated with COPP chemotherapy.