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
Q

TTP: px

A

purpura, fever, mental status changes, renal dysfunction, hematuria, hemolytic anemia

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26
Q

TTP: response to tx

A

80% response to medical therapy

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27
Q

TTP: tx

A

plasmapheresis (primary), immunosuppression … splenectomy rarely indicated

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28
Q

TTP: mortality

A

death MC 2/2 intracerebral hemorrhage or acute renal failure

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29
Q

post-splenectomy sepsis syndrome: occurrence rate

A

0.1% after splenectomy, inc risk in kids

30
Q

post-splenectomy sepsis syndrome: MC causes

A

strep pneumonia #1, H influenza, N meningitides

31
Q

post-splenectomy sepsis syndrome: pathophys

A

specific lack of immunity (immunoglobulin IgM) to capsulated bacteria

32
Q

post-splenectomy sepsis syndrome: highest risk patients

A

splenectomy for hemolytic disorders or malignancy

33
Q

post-splenectomy sepsis syndrome: mortality risk

A

kids have inc risk of mortality after developing PSSS

34
Q

post-splenectomy sepsis syndrome: mgmt. in kids

A

wait until at least 5yo before doing splenectomy b/c allows for Ab formation, child can get fully immunized

35
Q

post-splenectomy sepsis syndrome: when does this occur?

A

usually within 2 years of splenectomy

36
Q

post-splenectomy sepsis syndrome: post op mgmt. in kdis

A

kids <10yo should get ppx abx for 6 months (controversial)

37
Q

post-splenectomy sepsis syndrome: vaccines

A

BEFORE splenectomy give pneumococcus, meningococcus, H flu

38
Q

definition of hypersplenism

A

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
Q

hemolytic anemias: list

A

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
Q

hemolytic anemias: MC congenital type that leads to splenectomy

A

overall = spherocytosis …. non-membrane protein defect = pyruvate kinase deficiency

41
Q

hemolytic anemias: spherocytosis - describe, pathophys, result, mgmt

A

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
Q

hemolytic anemias: elliptocytosis - pathophys, px

A

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
Q

pyruvate kinase deficiency - epi, describe, pathophys

A

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
Q

G6PD deficiency - describe

A

hemolytic anemia 2/2 non-membrane protein defect …. precipitated by infection, certain drugs, fava beans … splenectomy usually NOT needed

45
Q

warm Ab-type acquired immune hemolytic anemia

A

hemolytic anemia 2/2 non-membrane protein defect …. indication for splenectomy

46
Q

sickle cell anemia - pathophys

A

hemolytic anemia 2/2 non-membrane protein defect …. HbgA replaced with HgbS —> spleen usually auto-infarcts and splenectomy NOT required

47
Q

beta thalassemia - pathophys, sx, mgmt., natural hx, tx

A

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
Q

Reed-Sternberg cells vs Howell-Jolly bodies

A

Hodgkin’s disease (fried eggs) vs hyposplenism (dark spot on RBC)

49
Q

Hodgkin’s disease: types

A

A - a-sx …. B - night sweats, fever, weight loss, unfavorable prognosis

50
Q

Hodgkin’s disease: stages

A

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
Q

Hodgkin’s disease: MC type

A

nodular sclerosing

52
Q

Hodgkin’s disease: prognosis

A

lymphocyte predominant = best prognosis vs lymphocyte depleted = worse prognosis

53
Q

Hodgkin’s disease: cells seen

A

Reed-Sternberg cells

54
Q

Hodgkin’s disease: tx

A

chemo

55
Q

Hodgkin’s disease: MCC of chylous ascites

A

lymphoma

56
Q

non-Hodgkin’s lymphoma: prognosis, MC type, dx, tx

A

worse than Hodgkin’s …. 90% B cell lymphoma …. usually systemic disease by the time the diagnosis is made …. tx w chemo

57
Q

hairy cell leukemia - tx

A

rarely need splenectomy

58
Q

spontaneous spleen rupture - occurs in what conditions?

A

mononucleosis, malaria, sepsis, sarcoid, leukemia, polycythemia vera

59
Q

splenosis

A

splenic implants, usually related to trauma

60
Q

hyposplenism

A

see Howell-Jolly bodies

61
Q

MC cause od splenic artery or splenic vein thrombosis

A

pancreatitis

62
Q

postsplenectomy changes

A

inc RBCs, inc WBCs, inc plts (if plt >1x10^6 then give ASA)

63
Q

MC splenic tumor - overall, benign, malignant

A

hemangioma is MC overall and benign … NHL is MC malignant

64
Q

splenic hemangioma mgmt

A

tx w splenectomy IF sx … (remember it is MC splenic tumor - overall and benign)

65
Q

NHL epi

A

MC malignant splenic tumor

66
Q

mgmt. of splenic cysts

A

surgery if sx or >10cm

67
Q

sarcoidosis of spleen - px and tx

A

anemia, dec plt …. tx = splenectomy for sx splenomegaly

68
Q

felty’s syndrome - describe, tx

A

rheumatoid arthritis, hepatomegaly, splenomegaly … tx = splenectomy for sx splenomegaly

69
Q

splenic abscess tx

A

usually splenectomy (bleeding risk w perc drainage)

70
Q

echinococcal splenic cyst tx

A

splenectomy

71
Q

results of splenectomy/hyposplenic condition - erythtocytes, plt, leukocytes

A

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
Q

guildelines for prevention of postsplenic sepsis

A

(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