This quiz contains recall questions from DM hematopathology PGIMER, entrance exam.
PART 1- 40 General pathology questions with a single option (No negative marking – 4 marks for a correct answer).
PART 2- 40 Hematopathology questions with multiple options. (Correct answer- four marks and wrong answer minus one mark).
HEMOPHILIA B LEYDEN is a rare form of FIX deficiency
Here are some key points to be noted regarding Hemophilia B Leyden.
- It is a rare form of FIX deficiency, hemophilia B Leyden, undergoes postpubertal phenotypic resolution.
- Patients with this condition present with hemophilia B in early childhood, with FIX activity ranging from less than 1% to 13% of normal.
- Plasma levels rise to as high as 70% of normal after the onset of puberty with resolution of bleeding complications.
- Mutations in hemophilia B Leyden have been identified in the promoter region of the FIX gene, within which a consensus sequence for steroid receptor binding is located.
REVIEW QUESTION- A QUESTION FROM DM HEMATOPATHOLOGY PGIMER ENTRANCE
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ANSWER is C: Steroid receptor is involved in the pathogenesis.