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34 Spleen Flashcards

1
Q

anatomy and physiology: arterial supply

A

short gastrics and splenic artery (end arteries), also some from L gastroepiploic

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2
Q

anatomy and physiology: venous drainage

A

splenic vein is posterior and inferior to splenic artery … drains to portal vein

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3
Q

anatomy and physiology: spleen function

A

antigen processing center for macrophages, largest producer of IgM

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4
Q

anatomy and physiology: tissue composition

A

85% red pulp = filter for aged or damaged RBCs (pitting and culling) … 15% white pulp = immunologic function, contains lymphocytes and macrophages

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5
Q

anatomy and physiology: Howell-Jolly bodies

A

nuclear remnants, removed by spleen (red pulp)

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6
Q

anatomy and physiology: Heinz bodies

A

Hb, removed by spleen (red pulp)

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7
Q

anatomy and physiology: describe function of red pulp

A

filter for aged or damaged TBCs … culling = removal of less deformable RBCs …. pitting = removal of abnormalities in RBC membrane (Howell-Jolly bodies = nuclear remnants, Heinz bodies = Hb)

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8
Q

anatomy and physiology: describe function of white pulp

A

immunologic unction, contains lymphocytes and macrophages, major site of bacterial clearance that lacks preexisting antibodies, site of removal of poorly opsonized bacteria/particles/cellular debris, antigen processing occurs with interactions between macrophages and help T cells

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9
Q

anatomy and physiology: tuftsin

A

opsonin, facilitates phagocytosis, produced in spleen

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10
Q

anatomy and physiology: poperdin

A

activates alternate complement pathway, produced in spleen

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11
Q

anatomy and physiology: hematopoiesis

A

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

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12
Q

anatomy and physiology: plt

A

spleen is reservoir

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13
Q

anatomy and physiology: accessory spleen

A

20%, MC found at splenic hilum

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14
Q

MC condition requiring splenectomy

A

ITP is the MC nontraumatic condition

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15
Q

indications for splenectomy

A

trauma, ITP, TTP (ITP much more common than TTP, ITP is MC non traumatic indication for splenectomy)

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16
Q

ITP: causes

A

drugs, viruses, etc

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17
Q

ITP: pathophys

A

anti-plt IgG –> bind plt –> decreased plt

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18
Q

ITP: px

A

petechiae, gingival bleeding, bruising, soft tissue ecchymosis

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19
Q

ITP: spleen abnl

A

spleen is normal

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20
Q

ITP: natural hx in kids

A

usually resolves spontaneously in kids <10 … AVOID splenectomy!

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21
Q

ITP: tx

A

steroids (primary therapy), gammaglobulin if steroid resistant, splenectomy indicated for those who fail steroids - removed IgG production and source of phagocytosis (80% respond after splenectomy), give platelets 1 hour preop

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22
Q

TTP: aka

A

thrombotic thrombocytopenic purpura

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23
Q

TTP: assoc with what?

A

medical reactions, infections, inflammation, autoimmune disease

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24
Q

TTP: pathophys

A

loss of plt inhibition —> thrombosis and infarction, profound thrombocytopenia

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25
TTP: px
purpura, fever, mental status changes, renal dysfunction, hematuria, hemolytic anemia
26
TTP: response to tx
80% response to medical therapy
27
TTP: tx
plasmapheresis (primary), immunosuppression ... splenectomy rarely indicated
28
TTP: mortality
death MC 2/2 intracerebral hemorrhage or acute renal failure
29
post-splenectomy sepsis syndrome: occurrence rate
0.1% after splenectomy, inc risk in kids
30
post-splenectomy sepsis syndrome: MC causes
strep pneumonia #1, H influenza, N meningitides
31
post-splenectomy sepsis syndrome: pathophys
specific lack of immunity (immunoglobulin IgM) to capsulated bacteria
32
post-splenectomy sepsis syndrome: highest risk patients
splenectomy for hemolytic disorders or malignancy
33
post-splenectomy sepsis syndrome: mortality risk
kids have inc risk of mortality after developing PSSS
34
post-splenectomy sepsis syndrome: mgmt. in kids
wait until at least 5yo before doing splenectomy b/c allows for Ab formation, child can get fully immunized
35
post-splenectomy sepsis syndrome: when does this occur?
usually within 2 years of splenectomy
36
post-splenectomy sepsis syndrome: post op mgmt. in kdis
kids <10yo should get ppx abx for 6 months (controversial)
37
post-splenectomy sepsis syndrome: vaccines
BEFORE splenectomy give pneumococcus, meningococcus, H flu
38
definition of hypersplenism
dec in circulating cell count of erythrocytes a/oplt a/o leukocytes AND normal compensatory heatopoietic responses present in bone marrow AND correction of cytopenia by splenectomy WITH OR WITHOUT splenomegaly
39
hemolytic anemias: list
membrane protein defects = spherocytosis, elliptocytosis ... non-membrane protein defects = pyruvate kinase deficiency, G6PD deficiency, warm Ab-type acquired immune hemolytic anemia, sickle cell, beta thal
40
hemolytic anemias: MC congenital type that leads to splenectomy
overall = spherocytosis .... non-membrane protein defect = pyruvate kinase deficiency
41
hemolytic anemias: spherocytosis - describe, pathophys, result, mgmt
membrane protein deficit .... MC congenital hemolytic anemia requiring splenectomy ... spectrin deficit (membrane protein) - deforms RBCs and leads to splenic sequestration (hypersplenism) ... causes pigmented stones, anemia, reticulocytosis, jaundice, splenomegaly .... tx = splenectomy (curative) and cholecystectomy (ideal to wait until >5yo before splenectomy, give immunizations first)
42
hemolytic anemias: elliptocytosis - pathophys, px
membrane protein deficit .... sx and mech similar to - spherocytosis, but elliptocytosis is less common .... spectrin AND PROTEIN 4.1 deficit - deforms RBCs and leads to splenic sequestration (hypersplenism) ... causes pigmented stones, anemia, reticulocytosis, jaundice, splenomegaly
43
pyruvate kinase deficiency - epi, describe, pathophys
hemolytic anemia 2/2 non-membrane protein defect .... results in congenital hemolytic anemia ... MC of this subtype of hemolytic anemia to result in splenectomy .... causes altered glucose metabolism, RBC survival is enhanced by splenectomy
44
G6PD deficiency - describe
hemolytic anemia 2/2 non-membrane protein defect .... precipitated by infection, certain drugs, fava beans ... splenectomy usually NOT needed
45
warm Ab-type acquired immune hemolytic anemia
hemolytic anemia 2/2 non-membrane protein defect .... indication for splenectomy
46
sickle cell anemia - pathophys
hemolytic anemia 2/2 non-membrane protein defect .... HbgA replaced with HgbS ---> spleen usually auto-infarcts and splenectomy NOT required
47
beta thalassemia - pathophys, sx, mgmt., natural hx, tx
MC thalassemia .... 2/2 persistent HbgF, major = btoh chain affected, minor = 1 chain affected and a-sx .... sx = pallor, retarded body growth, head enlargement .... most pts die in teens 2/2 hemosiderosis ... tx = medical - blood transfusions and iron chelators (deferoxamine, deferiprone), splenectomy (if pt has splenomegaly) may decrease hemolysis and sx
48
Reed-Sternberg cells vs Howell-Jolly bodies
Hodgkin's disease (fried eggs) vs hyposplenism (dark spot on RBC)
49
Hodgkin's disease: types
A - a-sx .... B - night sweats, fever, weight loss, unfavorable prognosis
50
Hodgkin's disease: stages
1 = 1 area or 2 contiguous areas on the same side of the diaphragm .... 2 = 2 non-contiguous areas on the same side of the diaphragm .... 3 = incolced on each side of diaphragm ... 4 = liver, bone, lung, or any other non-lymphoid tissue except spleen
51
Hodgkin's disease: MC type
nodular sclerosing
52
Hodgkin's disease: prognosis
lymphocyte predominant = best prognosis vs lymphocyte depleted = worse prognosis
53
Hodgkin's disease: cells seen
Reed-Sternberg cells
54
Hodgkin's disease: tx
chemo
55
Hodgkin's disease: MCC of chylous ascites
lymphoma
56
non-Hodgkin's lymphoma: prognosis, MC type, dx, tx
worse than Hodgkin's .... 90% B cell lymphoma .... usually systemic disease by the time the diagnosis is made .... tx w chemo
57
hairy cell leukemia - tx
rarely need splenectomy
58
spontaneous spleen rupture - occurs in what conditions?
mononucleosis, malaria, sepsis, sarcoid, leukemia, polycythemia vera
59
splenosis
splenic implants, usually related to trauma
60
hyposplenism
see Howell-Jolly bodies
61
MC cause od splenic artery or splenic vein thrombosis
pancreatitis
62
postsplenectomy changes
inc RBCs, inc WBCs, inc plts (if plt >1x10^6 then give ASA)
63
MC splenic tumor - overall, benign, malignant
hemangioma is MC overall and benign ... NHL is MC malignant
64
splenic hemangioma mgmt
tx w splenectomy IF sx ... (remember it is MC splenic tumor - overall and benign)
65
NHL epi
MC malignant splenic tumor
66
mgmt. of splenic cysts
surgery if sx or >10cm
67
sarcoidosis of spleen - px and tx
anemia, dec plt .... tx = splenectomy for sx splenomegaly
68
felty's syndrome - describe, tx
rheumatoid arthritis, hepatomegaly, splenomegaly ... tx = splenectomy for sx splenomegaly
69
splenic abscess tx
usually splenectomy (bleeding risk w perc drainage)
70
echinococcal splenic cyst tx
splenectomy
71
results of splenectomy/hyposplenic condition - erythtocytes, plt, leukocytes
erythrocytes = howell-jolly bodies (nuclear fragments, dark purple spot on RBC), Heinz bodies (Hb deposits, dark blue spot on RBC), pappenheimer bodies (Fe deposits, lots of dark speckles), target cells, spur cells (acanthocytes) .... plt = transient thrombocytosis .... leukocytes = transient leukocytosis, persistent lymphocytosis, persistent monocytosis
72
guildelines for prevention of postsplenic sepsis
(1) vaccinate with polyvalent pneumococcal vaccine at least 10-14 days BEFORE splenectomy if possible .... () if urgent splenectomy, wait at least 14 days postprocedure to vaccinate ... (3) for high risk pts (immunosuppressed, kids <10yo), give meningococcal vaccine and H flu vaccine .... (4) abx ppx for kids <5yo .... (5) early abx tx for initial signs of infection .... (6) MedicAlert bracelet

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