Chapter 34 Spleen

Front 1

Spleen serves as antigen-processing center for macrophages; largest producer of what antibody?

Back 1

IgM

Front 2

Acts as a filter for aged or damaged RBCs

Back 2

85% red pulp of the spleen

Front 3

Immunologic function; contains lymphocytes and macrophages
• Major site of bacterial clearance that lacks preexisting antibodies
• Site of removal of poorly opsonized bacteria particles, and cellular debris
• Antigen processing occurs with interaction btw macrophages and helper T-cells

Back 3

15% white pulp of the spleen

Front 4

an opsonin; facilitates phagocytosis produced in the spleen

Back 4

Tuftsin

Front 5

activates alternate complement pathway produced in spleen

Back 5

Properdin

Front 6

occurs in spleen before birth and in conditions such as myeloid dysplasia

Back 6

Hematopoiesis

Front 7

Most common location of Accessory spleen

Back 7

most commonly found at splenic hilum (20%)

Front 8

List the Indication for splenectomy

Back 8

-idiopathic thrombocytopenic purpura (ITP) far greater than for thrombotic thrombocytopenic purpura (TTP)
-Echinococcal cyst
-Hereditary spherocytosis (partial or complete)
-Splenic vein thrombosis
- ITP most common nontraumatic condition requiring splenectomy
-Warm antibody type-acquired immune hemolytic anemia
-pyruvate kinase deficiency

Front 9

List Hematological functions of the spleen

Back 9

-Culling or destruction of senescent erythrocytes
-Pitting or removal of cytoplasm inclusive in erythrocytes
-Reservoir for platelets and granulocytes
-Hematopoiesis as fetus or in conditions with bone marrow destruction

Front 10

List immunologic functions of the spleen

Back 10

-Filtration and trapping of circulatory antigens
-Lymphocyte stimulation and proliferation
Antibody production in germinal follicles
-Production of opsonin -tuftsin and properdin

Front 11

Name the disease
This can occur from many etiologies -drugs, viruses. etc.
• Antiplatelet antibodies IgG- bind platelets, cause decrease in platelets
• Petechiae, gingival bleeding, bruising, soft tissue ecchymosis
• Spleen is normal
• In children < 10 years, usually resolves spontaneously
• Tx: steroids (primary therapy), plasmapheresis, gamma globulin for steroid-resistant disease
• Splenectomy indicated for those who fail steroids > removes lgG production and
source of phagocytosis; 80% respond
-Give platelets 1 hour before surgery

Back 11

IDIOPATHIC THROMBOCYTOPENIC PURPURA

Front 12

Name the disease
• Associated w/ medical reactions, infections, inflammation, autoimmune disease
• Loss of platelet inhibition - leads to thrombosis and infarction, profound thrombocytopenia
• Purpura, fever, mental status changes, renal dysfunction, hematuria, hemolytic anemia
• 80% respond to medical therapy
• Tx: plasmapheresis (primary) , steroids, ASA
• Death most commonly due to intracerebral hemorrhage or acute renal failure
• Splenectomy rare

Back 12

THROMBOTIC THROMBOCYTOPENIC PURPURA

Front 13

Name the bacteria responsible for POST SPLENECTOMY SEPSIS SYNDROME

Back 13

S.pneumoniae (#1 ), H.influenzae, N.meningitidis

0.1% risk; increased risk in children

Front 14

What is etiology for post splenectomy sepsis syndrome?

Back 14

Secondary to specific lack of immunity (immunoglobulin, lgM) to capsulated bacteria

Front 15

List what Lab chages you would expect to see Postsplenectomy.

Back 15

increased RBCs, increased WBCs, increased platelets; if platelets > million, need ASA

Front 16

#1 splenic tumor overall;
#1 benign splenic tumor

Back 16

Hemangioma

• Splenectomy if symptomatic

Front 17

#1 malignant splenic tumor

Back 17

Non-Hodgkin's lymphoma

Front 18

When is sx indicated with Splenic cysts

Back 18

when cysts are greater than 10cm

Front 19

Def:
Decrease in circulating cell count of erythrocytes and/or platelets and/or leukocytes and Normal compensatory hematopoietic responses present in bone marrow and Correction of cytopenia by splenectomy with or without Splenomegaly

Back 19

Hypersplenism

Front 20

Condition affecting the spleen, associated with increased venous pressure ( portal hypertension, CHF), malignant disease (leukemia), chronic inflammatory disease (Felty's syndrome,
SLE, sarcoidosis), myeloproliferative disease, infectious disease, amyloidosis, AIDS,
hemolytic anemias, polycythemia vera

Back 20

Secondary hypersplenism (most common)

Splenectomy may be indicated for symptomatic hypersplenism

secondary much more common then primary

Front 21

Disease that may cause secoundary hypersplenism
rheumatoid arthritis, hepatomegaly, splenomegaly
• Tx: splenectomy for symptomatic splenomegaly

Back 21

Felty's syndrome

Front 22

lipid metabolism disorder leading to splenomegaly
• Partial splenectomy may be effective

Back 22

Gaucher's disease

Front 23

• Spectrin deficit ( membrane protein) deforms RBCs and leads to splenic sequestration
• Causes pigmented stones, anemia, reticulocytosis, jaundice, splenomegaly
• Try to perform splenectomy after age 5 ; give immunizations first
• Tx: splenectomy and cholecystectomy
• Splenectomy curative

Back 23

Spherocytosis

Front 24

• Most common congenital hemolytic anemia requiring splenectomy

Back 24

Spherocytosis

Front 25

• Symptoms and mechanism similar to spherocytosis; less common
• Spectrin and protein 4.1 deficit (membrane protein)

Back 25

Elliptocytosis

Front 26

HEMOLYTIC ANEMIAS - NON-MEMBRANE PROTEIN DEFECTS
• Results in congenital hemolytic anemia
• Causes altered glucose metabolism; RBC survival enhanced by splenectomy

Back 26

pryruvate kinase deficiency

Front 27

Most common congenital hemolytic anemia not involving a membrane Protein that
requires splenectomy

Back 27

pryruvate kinase deficiency

Front 28

HEMOLYTIC ANEMIAS - NON-MEMBRANE PROTEIN DEFECTS
Precipitated by infection, certain drugs, fava beans
• Splenectomy usually not required

Back 28

G6PD deficiency

Front 29

HgbA replaced with HgbS
• Spleen usually autoinfarcts and splenectomy not required

Back 29

Sickle cell anemia

Front 30

• Major - both chains affected ; minor - 1 chain , asymptomatic
• Symptoms: pallor, retarded body growth, head enlargement
• Persistent HgbF
Splenectomy may decrease hemolysis and symptoms
• Most die in teens secondary to hemosiderosis

Back 30

Beta thalassemia

Front 31

List the stages of Hodgkins disease

Back 31

• A - asymptomatic
• B - symptomatic (night sweats, fever, weight loss) >unfavorable prognosis
• Stage I - 1 area or 2 contiguous areas on the same side of diaphragm
• Stage II - 2 noncontiguous areas on the same side of diaphragm
• Stage Ill - involved on each side of diaphragm
• Stage IV - liver, bone, lung, or any other nonlymphoid tissue except spleen

• Lymphocyte predominant - best prognosis
• Lymphocyte depleted - worst prognosis
• Nodular sclerosing - most common

Front 32

Tx for HODGKIN'S DISEASE

Back 32

• Tx: XRT and chemotherapy with vincristine, cyclophosphamide, prednisone, procarbazine

Front 33

MCC of Chylous ascites

Back 33

lymphoma

Front 34

• Gen erally system i c d isease by the ti m e the d i agnosis is made
• 90% are B-cell lymphomas
• Tx: XRT and chemotherapy with vincristine, cyclophosphamide, prednisone, Adriamycin

Back 34

NON-HODGKIN'S LYMPHOMA

Front 35

What is the tx for Hairy cell leukemia

Back 35

splenectomy, INF-y

Front 36

Classic pentad for TTP

Back 36

1. low platelets
2. Mental Status Change
3. Kidney failure
4. Fever
5. Microangiopathic hemolytic anemia

Front 37

Tx for TTP

Back 37

Plasmapheresis- gets rid of vWF molecules

Front 38

Dz: arise from deficiency or inhibition of enzyme ADAMTS13 (which is responsible for cleaving large vWF molecules)

Back 38

Thrombotic Thrombocytopenia Purpura

Front 39

MCC of chylous ascites

Back 39

Lymphoma

Front 40

4 cardinal signs of hypersplenism

Back 40

1. cytopenia
2. compensatory response to cytopenia in bone marrow
3. splenomegaly
4. correction of cytopenia following splenectomy