This 16 yr male boy came to our opd with severe pallor with splenohepatomegaly ( spleen 8cm bcm liver 4 cm bcm) with off and on fever negative for malaria and kala azar( K 39) what could be possible diagnosis and management pt is very poor we can't afford many investigation no prior transfusion history
Exclude mainly sickle cell anaemia, and other hemolytic anaemia. We can exclude thalassemia by his age and with out previous history of blood transfusion. . Tlc is only 4700 /cmm so we can exclude cml I think pt can afford for sickling TEST and comment on peripheral smear by a pathologist
We see lot of sickle cell anemia in our region and many come with huge splenomegaly, so auto splenectomy is not universal. I shall do a hemoglobin electrophoresis, and if the patient can not afford, send him to nearest medical college where it can be done, it looks like hemolytic anemia and a sickle thal double heterozygous is very likely
Splenohepatomegaly with severe anemia Hemolytic facies+,PS shows anisopoikilocytosis,microcytic hypochromic,Indirect hyper bilirubinemia,Gall stone on USG.All points towards hemolytic anemia most probably thalassemia.Reticulocyte is not much raised compared to anemia. Ask for any family history of BT,anemia,patiet is from which place and community? Do HPLC to confirm the diagnosis.
Spleenomegally excludes sickle cell anaemia. Hb electopheresis to exclude thalassemia intermedia ( most probable diagnosis. Serological test/ spleen fnac to exclude Kalaazar.
Malaria cmll thalasemia lymphoproliferative disease histocytosis x
Chronic hemo, lytic anaemia. Do HPLC, iron study, bone marrow study.
Family history.any h/o blood transfusion.Increase ALKALINE phosphatase may be due to cholelithiasis causing obstructive jaundice.HPLC to rule out haemoglobinopathy/sickle cell disease.
HB electrophoresis to rule out hemolytic anemia... Tsb is raised.. hemolytic facies can be seen
Get CBC with pbf done.Just one investigation that will clinch the diagnosis, I.e bone marrow aspiration and biopsy alonwith ldh, uric acid, calcium. Bcoz there's a high possibility of lymphoproliferative malignancy which we can't afford to miss.
Hemolytic anemia - thaassemia
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