Chapter 34 - Spleen

Question 1

Where is the splenic v in relation to splenic a?

Answer 1

Posterior and inferior

Question 2

Spleen is the largest producer of what antibody?

Answer 2

IgM

Question 3

What % of the spleen is red pulp? Function?

Answer 3

85%; acts as filter for aged or damaged RBCs

Question 4

What is pitting?

Answer 4

Removal of abnormalities in RBC membrane

Question 5

What are Howell-Jolly bodies?

Answer 5

Nuclear remnants

Question 6

What are Heinz bodies?

Answer 6

Hemoglobin

Question 7

What is culling?

Answer 7

Removal of less deformable RBCs

Question 8

What % of the spleen is white pulp? Function?

Answer 8

15%; immunologic function, contains lymphocytes and macrophages; major site of bacterial clearance that lacks preexisting abs; site of removal of poorly opsonized bacteria/debris

Question 9

What is tuftsin?

Answer 9

An opsonin; facilitates phagocytosis (produced in spleen)

Question 10

What is properidin?

Answer 10

Activates alternate complement pathway (produced in spleen)

Question 11

When does hematopoiesis occur in the spleen?

Answer 11

Before birth and in conditions such as myeloid dysplasia

Question 12

Where is the most common location of accessory spleen?

Answer 12

Splenic hilum (20%)

Question 13

What is the most common nontraumatic condition requiring splenectomy?

Answer 13

ITP

Question 14

What happens in ITP?

Answer 14

Antiplatelet IgG binds platelets, cause dec. platelet count –> gingival bleeding, petechiae, bruising, soft tissue ecchymosis

Question 15

Treatment for ITP in children? Adults?

Answer 15

Children <10, usually resolves spontaneously; steroids (primary therapy), plasmapheresis, gammaglobulin for steroid-resistant disease, splenectomy for those who fail steroids

Question 16

Why does splenectomy fix ITP?

Answer 16

Spleen is normal; removes IgG production and source of phagocytosis (80% respond)

Question 17

TTP associated with what conditions?

Answer 17

Medial reactions, infections, inflammation, autoimmune disease

Question 18

What happens in TTP?

Answer 18

Loss of platelet inhibition leads to thrombosis and infarction, profound thrombocytopenia; purpura, fever, AMS, renal dysfunction, hematuria, hemolytic anemia

Question 19

Treatment for TTP?

Answer 19

Plasmapheresis (primary), steroids, ASA - 80% respond to medical therapy

Question 20

Most common cause of death due to TTP?

Answer 20

Due to ICH or acute renal failure

Question 21

Risk of postsplenectomy sepsis?

Answer 21

0.1%, increased risk in children; highest in pts with splenectomy due to hemolytic disorders or malignancy

Question 22

Most common bacteria causing OPSS?

Answer 22

S. pneumonia, N. meningitidis, H. flu

Question 23

Most episodes occur within what time frame?

Answer 23

2y post-splenectomy

Question 24

Ideally how old should a child be before splenectomy?

Answer 24

5; allows Ab formation, child can get fully immunized

Question 25

Vaccines needed before splenectomy?

Answer 25

Pneumococcus, meningococcus, H. flu

Question 26

Postsplenectomy lab changes?

Answer 26

Increased RBCs, WBCs, platelets; if plt >1x10^6, need ASA

Question 27

What is the #1 splenic tumor overall as well as the #1 benign splenic tumor? Treatment?

Answer 27

Hemangioma; splenectomy if symptomatic

Question 28

What is the #1 malignant splenic tumor?

Answer 28

Non-Hodgkin's lymphoma

Question 29

When is operation indicated for splenic cyst?

Answer 29

If symptomatic or >10cm

Question 30

Lab changes in hypersplenism?

Answer 30

Decreased platelets, WBCs, and RBCs

Question 31

What is the definition of hypersplenism?

Answer 31

Dec. in circulating RBC, plts, or WBC AND normal compensatory hematopoietic response in bone marrow AND correction of cytopenia by splenectomy W/ or W/OUT splenomegaly

Question 32

What is the most common cause of hypersplenism?

Answer 32

Secondary: increased venous pressure (portal HTN, CHF), malignant disease, chronic inflammatory disease, myeloproliferative disease, infectious, amyloidosis, AIDS, hemolytic anemias, polycythemia vera

Question 33

Sarcoidosis of the spleen causes what?

Answer 33

Anemia, decreased platelets

Question 34

What is Felty's syndrome?

Answer 34

RA, hepatomegaly, splenomegaly

Question 35

What is Gaucher's disease?

Answer 35

Lipid metabolism disorder leading to splenomegaly

Question 36

What is the most common congenital hemolytic anemia requiring splenectomy?

Answer 36

Spherocytosis

Question 37

What is the defect in spherocytosis?

Answer 37

Spectrin deficit (membrane protein) causing deformed RBCs and splenic sequestration

Question 38

What does spherocytosis lead to?

Answer 38

Pigmented stones, anemia, reticulocytosis, jaundice, splenomegaly

Question 39

Treatment for spherocytosis?

Answer 39

Splenectomy and cholecystectomy

Question 40

What is elliptocytosis?

Answer 40

Spectrin and protein 4.1 deficit (membrane protein); causes symptoms similar to spherocytosis

Question 41

What does pyruvate kinase deficiency cause?

Answer 41

Results in congenital hemolytic anemia; altered glucose metabolism, RBC survival enhanced by splenectomy

Question 42

What is the most common congenital hemolytic anemia NOT involving a membrane protein deficit?

Answer 42

Pyruvate kinase deficiency

Question 43

What precipitates hemolysis with G6PD deficiency?

Answer 43

Infection, certain drugs, fava beans; splenectomy usually not required

Question 44

Is splenectomy required for sickle cell anemia?

Answer 44

Spleen autoinfarcts, splenectomy NOT required

Question 45

Use of splenectomy in beta thalassemia?

Answer 45

May decrease hemolysis and symptoms; most die in teens secondary to hemosiderosis

Question 46

What cells are characteristic of Hodgkin's disease?

Answer 46

Reed-Sternberg cells

Question 47

How is Hodgkin's disease staged?

Answer 47

Stage I: 1 area or 2 contiguous areas on same side of diaphragm; stage II: 2 noncontiguous areas on same side of diaphragm; stage III: involved on each side of diaphragm; stage IV: liver, bone, lung or any other nonlymphoid tissue except spleen

Question 48

What are the different types of Hodgkin's lymphoma? What has the best prognosis? Most common?

Answer 48

Lymphocyte predominant (best prognosis), lymphocyte depleted (worst prognosis), nodular sclerosing (most common)

Question 49

What is the most common cause of chylous ascites?

Answer 49

Lymphoma

Question 50

What % of non-Hodgkin's lymphomas are B cell?

Answer 50

90%

Question 51

Treatment for hairy cell leukemia?

Answer 51

Splenectomy, INF-gamma

Question 52

What are the causes of spontaneous splenic rupture?

Answer 52

Mononucleosis, malaria, sepsis, sarcoid, leukemia, polycythemia vera

Question 53

What is splenosis? Cause?

Answer 53

Splenic implants; usually related to trauma

Question 54

When do you see Howell-Jolly bodies?

Answer 54

In hyposplenism

Question 55

What is the most common cause of splenic artery or splenic vein thrombosis?

Answer 55

Pancreatitis

Question 56

What patients get splenic artery aneurysms?

Answer 56

Females; secondary to fibromuscular dysplasia, atherosclerosis

Question 57

What are pappenheimer bodies?

Answer 57

Iron deposits