Trastornos mieloproliferativos y leucemia en síndrome de Down. Presentación de dos casos clínicos en el período neonatal / Myeloproliferative disorders and leukemia related to Down syndrome. Report of two clinical cases in neonatal period

ElsíndromedeDownpredisponeatrastornosmieloproliferativos. Se estima que del 5 % al 30 % de los neonatos con esta condición desarrollarán mielopoyesis anormal transitoria. El tratamiento no está estandarizado; la exanguinotransfusión y la citarabina podrían ser efectivos. Se describen dos casos de pacientes con síndrome de Down, quienes durante el período neonatal presentaron leucemia mieloide aguda y mielopoyesis anormal transitoria, los tratamientos utilizados y sus desenlaces. Se considera que la sospecha y el diagnóstico temprano de esta entidad son factores determinantes en el pronóstico.

Down syndrome predisposes to haematological disorders. It is estimated that 5-30% of neonates with this condition will develop transient abnormal myelopoiesis. Treatment is not standardized; exchange transfusion and the use of cytarabine could be effective. We present two clinical cases of patients with Down syndrome, who during the neonatal period showed acute myeloid leukemia and transient abnormal myelopoiesis, the treatments used and their outcomes. Suspicion and early diagnosis of this entity are considered determining factors in prognosis.

A case of leukemoid reaction in a patient with sarcomatous hepatocellular carcinoma / 대한간학회지

A leukemoid reaction is defined as reactive leukocytosis exceeding 50,000/mm3, with a significant increase in early neutrophil precursors, and can be a paraneoplastic manifestation of various malignant tumors. A 71-year-old male patient complained of decreased appetite, fatigue, and abdominal fullness. He had a palpable, firm liver, and laboratory investigations suggested leukemoid reaction. Liver dynamic computed tomography revealed a hypervascular mass, and an ultrasound-guided fine-needle aspiration of the mass confirmed hepatocellular carcinoma (HCC) with a sarcomatoid component. The leukocyte count of the patient had increased to 147,800/mm3, and he died 10 days after admission. This is a rare case of leukemoid reaction in a patient with sarcomatous HCC.

Leukocyte adhesion deficiency type 1 presenting as leukemoid reaction.

Leukocyte Adhesion Deficiency (LAD) is characterized by the inability of leukocytes, in particular neutrophilic granulocytes, to emigrate from the blood stream towards sites of inflammation. Infectious foci are nonpurulent and may eventually become necrotic because of abnormal wound healing. LAD-I is characterized by the absence of the beta-2 integrins CD II and CD 18 on leukocytes. We present a male neonate referred with continuation of leukemoid reaction in spite of antibiotic therapy of simultaneous omphalitis. The diagnosis of LAD-I was confirmed by flow cytometry, showing a deficiency of beta-2 integrins on the surface of the leukocytes. After management, the patient was discharged with prophylactic antibiotics.

Hepatocellular carcinoma presenting as neutrophilic leukaemoid reaction--a rare entity.

Leukaemoid reaction is a rare, growth factor-driven, paraneoplastic manifestation of hepatocellular carcinoma. It may masquerade as the neutrophilic chronic myeloid leukaemia or as chronic neutrophilic leukaemia. A 52-year-old male presented with hepatosplenomegaly and severe leucocytosis. He had progressive leucocytosis, neutrophil alkaline phosphatase score elevated, liver function tests altered. FNAC from the mass in the liver revealed features of moderately differentiated hepatocellular carcinoma. The patient deteriorated within two weeks and died thereafter.

Unusual presentation of amoebic liver abscess as myeloid leukemoid reaction

A case of amoebic liver abscess presenting as myeloid Leukemoid reaction in a forty year male is reported. The presenting white cell count was 144.8 x 10 / I and peripheral smear mimicked chronic myeloid leukemia - chronic phase. Treatment with metronidazole resulted in complete clinical and haematological cure. The recognition of the condition presenting as myeloid Leukemoid reaction is stressed in an endemic setting

Aspectos básicos da contribuiçäo das reaçöes leucemóides para o diagnóstico em medicina interna / Basic aspects of the contribution in internal medicine diagnosis

Os autores apresentam a revisao das bases clínicas e laboratoriais das reaçoes leucemóides, destacando sua importância no encaminhamento da definiçao diagnóstica no universo da Medicina Interna.

Trastorno mieloproliferativo transitorio asociado a síndrome de Down en recién nacidos / Transient myeloproliferative disorders associated with Down's syndrome in newborn infants

Se presenta cuatro casos de reacción leucemoide transitoria asociada a síndrome de Down (RLTAD) en recién nacidos. En todos los pacientes el diagnóstico se planteó en la primera semana de vida por los resultados del hemograma. El cuadro hematológico se caracterizó por leucocitosis de 15.000 a 48.000 glóbulos blancos-mm3 con 20 a 60% de mieloblastos circulantes y normalidad de las series eritrocitaria y plaquetaria. En tres casos había evidencia de hepatomegalia. La evolución clínica y hematológica se caracterizó por desaparición de los blastos circulantes en un plazo entre 1 y 4 meses, en coincidencia con la desaparición de la hepatomegalia. Dos pacientes presentaron una evolución clínica posterior muy favorable con controles hematológicos normales a los tres años de observación. Los otros dos fallecieron cuando la reacción leucemoide había desaparecido, a causa de infecciones graves como las que se asocian frecuentemente al síndrome de Down

Reacción leucemoide en recién nacido de pretérmino por uso de corticosteroides en periodo antenatal / Leukemoid reaction in newborn preterm for use of costicosteroides in prenatal period

Se presenta el caso de un recién nacido de pretérmino de muy bajo peso al nacer, que presentó una reacción leucemoide secundaria a la administración antenatal de dexametasona. Se discuten las características hematológicas y clínicas del caso, así como la patogénesis propuesta. (Rev. Cost. Cienc. Méd. 1989; 10(1): 43-7)