RESUMEN
Sickle Cell Disease (SCD) is a structural haemoglobinopathy which is extremely diverse in its presentation regarding disease severity and organ involved. The homozygous form if poorly managed gives rise to numerous life threatening conditions which are otherwise avoidable. Here we report the case of a male adolescent with homozygous SCD who presented with haemolytic anaemia, massive ascites, hepatomegaly and multiple fractures secondary to severe malnourishment associated with the disease.
Asunto(s)
Anemia de Células Falciformes , Ascitis , Hepatomegalia , Fracturas del Húmero , Adolescente , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/fisiopatología , Ascitis/complicaciones , Ascitis/diagnóstico , Ascitis/terapia , Hepatomegalia/complicaciones , Hepatomegalia/diagnóstico , Hepatomegalia/patología , Humanos , Fracturas del Húmero/complicaciones , Fracturas del Húmero/diagnóstico por imagen , Fracturas del Húmero/terapia , Masculino , PakistánRESUMEN
BACKGROUND: Hodgkin's lymphoma (formerly, Hodgkins disease) is a potentially curable malignancy with distinctive biological behavior and specific clinical characteristics. Limited information is available from developing countries for patients with classical Hodgkin's lymphoma (cHL). Therefore we reviewed the demographical and clinico-hematological profiles along with bone marrow infiltration patterns in adult patients presenting at Liaquat National Hospital and Medical College. MATERIALS AND METHODS: In this cross sectional study, 62 adult (≥15 years) patients with cHL were enrolled from January 2010 to December 2014. RESULTS: The mean age was 29.7±13.8 years with a median of 30 years. The male to female ratio was 2:1. B symptoms were present in 72.5% of patients and lymph node enlargement in 85.4%. The frequency of bone marrow infiltration in our cHL patients was found to be 27.4%, the pattern being predominantly focal followed by diffuse. The mean hemoglobin was 9.4±1.9g/dl with a mean MCV of 78.1±7.9 fl, a mean total leukocyte count of 10.9±20.6x109/l and a mean platelet count of 241.6±150.1x109/l. CONCLUSIONS: Our analysis shows that clinico-pathological features of cHL in Pakistan are comparable to published data. Peripheral lymphodenopathy associated with B symptoms is the commonest presentation. Bone marrow involvement is more common in our setup as patients usually presented at an advanced stage of disease.
