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  • 2. A 12 yr old girl presents with progressive pallor, lethargy and fatiguability. On examination apart from pallor nothing significant. Her CBC shows a pancytopenia. a) What is the bone marrow diagnosis? b) Which invest. will you ask for? Q1
  • 3. ANSWERS A) Megaloblastic anaemia B) CBC/PS Bone marrow Serum B12 level Serum And Red Cell folate Levels Urinary Excretion Of Orotic Acid To Exclude Orotic Aciduria Deoxy Uridine Supression Test to Dicrminate between Folate and Cobalamine Deficiencies
  • 4. A) Identify the abnormality on peripheral smear? B) Name 4 conditions where these cells can be seen. Q2
  • 5. • 2 A) Target cell • B) Thalassemia • Hemoglobinopathies • Hb AC or CC • Hb SS, SC, S-thal • Liver Disease • Post Splenectomy or Hyposplenic states • Severe Iron deficiency • HbE(Hetro and Homozygous) • LCAT deficiency • A betalipoprotenemia
  • 6. A 10 yr old girl ,having ITP since 6 months has ecchymotic patch on her leg and has uncontrollable epistaxis. Her platelet count is 4000. A) Which 3 invest. would you advice in her? B) What 4 measures either singly or in combination can be used to treat life threatenig haemorrhage in ITP? Q3
  • 7. • 3A) ANA, ds DNA • HIV, • Ser. Immunoglobulin • 3B) Platelet transfusion • Methyl Prednisolone – 500mg/m2 IV /day x 3 days • IV Ig 0.8 gm/kg • Emergency splenectomy
  • 8. A 10 yr old boy with fever and cough since 3 months, breathlessness since 15 days is being treated with anti tuberculous drugs since the last 1.5 months without relief. A) Give the differential diagnosis of mediastinal widening in anterior, middle and posterior mediastinum? Q4
  • 9. • 4) Anterior medistinum----- – Lymphoid tumors,NHL,HD,ALL – Thyroid tumors – Thymoma – Bronchogenic cysts, Aneurysm, Lipoma • Middle Mediastinum – LN Masses – Tertoma – Angioma – Diaphargmatic hernia, Pericardial cyst, Bronchogenic Cyst, Esophageal lesions, • Posterior mediastinum – Neurologic tumors(neuroblastoma, ganglioneuroma) – Lymphoma – RMS – Ewing’s sarcoma – Bronchogenic Cysts, Enterogenous cysts, Thoracic meningocele
  • 10. A) Which haematological condition is this boy with short stature and double thumb suffering from? B) What is the inheritance pattern? C) Which is the commonest haematological malignancy these children can develop? Q5
  • 11. • 5 A) Fanconis Anemia • B) autosomal recessive • C) MDS &/or AML
  • 12. A) Which 1 inves would you advice? B) What is the diagnosis? C) Which type would he be categorized as? D) What is the mode of inheritance? 1.5yr old boy with minor injury develops this swelling? Q6
  • 13. • A) PT/PTTK • B) hemophillia A or B • C) moderate • D) X linked Recessive
  • 14. A) Identify the abnormality on peripheral smear? B) What is the inheritance pattern? Q7
  • 15. • 7 A) Sickle Cells • B) Autosomal dominant or spontaneous mutation
  • 16. A) Name the tumor and the eye. B) Name any 2 conditions associated with this tumor. This 3 yr old girl with a solid tumor has developed a periorbital haemorrhage. Q8
  • 17. • 8 A)Neuroblastoma and Raccoon eyes • B) Neurofibromatosis • Hirschprung’s Disease with aganglionic colon • Phaeochromocytoma in family • Fetal Hydantoin syndrome • Fetal alcohol syndrome • Nesidioblastosis
  • 18. A) Identify the abnormality on the peripheral smear? B) Name 4 conditions where this smear can be seen? Q9
  • 19. • 9 A) Spherocytes • B) Hereditary spherocytosis, • Burns, • AIHA, • ABO incompatibility • Microangiopathic hemolytic anemia • SS Disease • Hypersplenism • Post transfusion • PK deficiency • Water dilution hemolysis
  • 20. A) What is the bone Marrow Diagnosis? B) Give 5 poor prognostic factors associated with this condition? 11 yr old male child presents with pallor, fatigue and hepatosplenomegaly.His Cbc shows pancytopenia Q10
  • 21. • 10 A) AML • B) WBC > 100000/cumm • Monosomy 7 • Secondary AML • FLT3 ITD • MRD present after induction
  • 22. A) What is the most likely diagnosis? B) Which 2 investigations will clinch the diagnosis? C) What is the treatment of this condition? A 3 month old infant exclusively breast fed presents with loose motions since early neonatal period. On examination she is pale and is failing to thrive. Her CBC shows a pancytopenia. Her blood sugar is 200mg/dl. Q11
  • 23. • 11 A) Pearson marrow pancreas Syndrome • B) Macrocytic anemia with increased HbF • Ringed sideroblasts with vacuolization of marrow precursors (erythroid and granulocytic series) • C) packed cell with G CSF transfusion.
  • 24. A 4 day old neonate with birth asphyxia was referred to the haematology services for a very high WBC count in the range of 60,000, the platelet count being 6 lakhs. Sepsis in the newborn was ruled out. The baby was cyanosed on examination. A)What is the most likely diagnosis? B)Is the condition primary or secondary? C)Name tests to be performed to prove your diagnosis? Q12
  • 25. • 12)a) Polycythaemia vera • B) secondary • C) CBC including differential WBC • To diagnose secondary polycythaemia--- • Arterial O2 saturation • Carboxyhaemoglobin • Renal USG • Abdominal/ Cranial CT • Special studies for polycythaemia vera • leucocyte alkaline phosphatase • B12/B12 binding capacity • Erythropoeitin level • RBC colony formation
  • 26.