Chapter 34 - Spleen++

Question 1

Where is the splenic v in relation to splenic a?

Answer 1

Posterior and inferior. It is behind the pancreas.

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” caused by spleen seen on path slides for RBC?

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 in regards to spleen function?

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. Medical indications are now greater cause for splenectomy than trauama.

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… meds first, then surgery if that fails.

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

Medical 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. ADAMST13 association.

Question 19

Treatment for TTP?

Answer 19

Plasmapheresis (primary), steroids, ASA - 80% respond to medical therapy. No defined role for surgery.

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 of OPSS 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. Pancytopenia.

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 (SLE), 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 (HS)

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 (lacunar histiocytes)

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

purine analog meds; splenectomy if symptomatic splenomegaly

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

Question 58

Embryology of spleen

Answer 58

mesoderm, capillary network develops in 30 days, B&T cells show up in 13 weeks, IgG and IgM available in 3rd trimester

Question 59

What do you suspect if a patient with hematologic disease has persistence after splenectomy?

Answer 59

accessory or acquired splenosis

Question 60

What is a feared complication after splenectomy that can demonstrate itself as sepsis w/ an intra-abdominal fluid collection?

Answer 60

pancreatic injury - drain, abx

Question 61

What is the most common visceral artery aneurysm?

Answer 61

splenic artery aneurysm

Question 62

If you are mobilizing the splenic flexure, what must you mutch for near the spleen?

Answer 62

inferior pole arteries - can cause problematic bleeding

Question 63

What extra medication must be given to young children s/p splenectomy?

Answer 63

penicillin for PNA ppx x2 years

Question 64

How do you treat a splenic abscess?

Answer 64

splenectomy and abx

Question 65

When does OPSI usually occur?

Answer 65

within the first 2 years post-splenectomy

Question 66

Who is at greatest risk of getting OPSI?

Answer 66

Postsplenectomy sepsis is greatest in patients with underlying hematologic conditions. The risk in children is higher than in adults. Sepsis is seen particularly in children younger than 15 years of age, mostly in those younger than 5.

Question 67

In pts w/ G6PD def, what are some triggers for anemia?

Answer 67

Infection is common. Also sulfonamides, fava beans, antimalarials, and nitrofurantoin.

Question 68

Hypotension, abdominal tenderness, and splenomegaly are common symptoms of what process?

Answer 68

Hypersplenism

Question 69

After partial splenectomy, at what portion of remaining spleen can function be expected to be maintained (not requiring postop vaccination)?

Answer 69

Splenic function is preserved in patients with more than one-third of the spleen remaining.

Question 70

What initial tests are helpful in determining accessory/remnant spleen in patients s/p splenectomy who have recurrent symptoms?

Answer 70

Radionucleotide imaging and blood smear. | Expect to see spleen tissue in hilum and lack of Howell-Jolly bodies on blood smear.