Pathology 4

Question 1

Child with lyticc bone lesions and skin rash or recurrent otitis media with a mass involving mastoid bone

Answer 1

Langerhans Histiocytosis

Question 2

Birbeck Granules

Answer 2

Tennis rackets on EM, Langerhans Histiocytosis

Question 3

Letterer-Siwe Disease

Answer 3

Malignant proliferation of Langerhans cells, skin rash and cystic skeletal defects in infant <2y/o, multiple organs and rapidly fatal

Question 4

Eosinophilic Granuloma

Answer 4

Benign proliferation of Langerhans cells in bone. Fz in adolescent, no rash. Bx with inflammation and EOSINOPHILS

Question 5

Hand-Schuller-Christian Disease

Answer 5

Malignant proliferation of Langerhans, scalp rash, lytic skull defects, diabetes insipidus and exopthalmos in child (kids >3y/o)

Question 6

Mutation in JAK2

Answer 6

Polycythemia Vera

Question 7

Intense Itching After a hot shower

Answer 7

Polycythemia Vera

Question 8

Tx of Polycythemia Vera

Answer 8

Phlebotomy and secondarily use Hydroxyurea

Question 9

Complications of Polycythemia Vera

Answer 9

Urecemia and gout

Question 10

Enlarged Megakaryocytes, mutation in JAK 2 kinase, Overproduction of abnormal platelets

Answer 10

Essential Thrombocytosis

Question 11

Fibrotic Obliteratino of BM , Teardrop RBCs and immature forms of myeloid line

Answer 11

Myelofibrosis

Question 12

Relative Polycythemia

Answer 12

decreased plasma volume from dehydration or burns

Question 13

Appropriate Absolute Polycythemia

Answer 13

lung disease, congenital heart disease, high altitude

Question 14

Inappropriate absolute polycythemia

Answer 14

renal cell carcinoma, Wilms tumor, cyst, hepatocellular carcinoma, hydronephrosis, due to ECTOPIC EPO

Question 15

Who is a stupid clinical coordinator

Answer 15

Rita