Term
| % of spleen that is red pulp? |
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Definition
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Term
Pitting
-what are the RBC remnants and what are they composed of? |
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Definition
1. Howell-Jolly = nuclear remnant
2. Heinz body = Hgb
3. Pappenheimer bodies = Iron |
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Term
| Role of red vs white pulp? |
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Definition
| pitting vs immune function |
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Term
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Definition
-removal of poorly opsonized bacteria, particels, cellular debris
-Ag processing for dentritic cells/Mphages
-Largest IgM producer |
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Term
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Definition
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Term
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Definition
-an opsonin produced in spleen
-activates alt complement pathway |
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Term
| MC accessory spleen location? |
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Definition
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Term
| MC non traumatic reason for splenectomy? |
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Definition
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Term
post-splenectomy changes
-labs
-mgmt? |
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Definition
-Inc RBC, WBC, PLT
-remnants
-Spur and target cells
**ASA if PLT >1 mil** |
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Term
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Definition
-hemangioma (MC benign and overall)
-NHL = MC malig |
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Term
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Definition
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Term
| when to operate on splenic cyst? |
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Definition
| if > 10 cm (due to CA risk) |
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Term
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Definition
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Term
Immune Thrombocytopenic Purpura
-Sx |
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Definition
| purpura, petechiae, gingival bleeds, brusing |
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Term
Immune Thrombocytopenic Purpura
-path |
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Definition
normal spleen, low PLT
-2/2 antiPLT IgG binds PLT-->splenic destruction by macrophages |
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Term
| cause of Immune Thrombocytopenic Purpura in kids? |
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Definition
usually viral
90% spont res |
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Term
Immune Thrombocytopenic Purpura
-initial tx |
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Definition
1. steroids (Gammaglob vs resistant dz)
2. RhoGAM if Rh+ve (competes for Mphage binding sites and prevents PLT destruction)
3. keep PLT > 20K (but avoid giving PLT) |
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Term
Immune Thrombocytopenic Purpura
-acute bleed mgmt |
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Definition
steroids (MePred 1gm/d)
-IV-IG
*might need PLT transfusion, but try to avoid* |
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Term
Immune Thrombocytopenic Purpura
-Refractory tx options |
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Definition
Rituximab (anti CD20, destroys B cells)
-azathioprine, cyclophosphamide
**SPLENECTOMY = 80% response** |
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Term
| indications for splenectomy vs ITP? |
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Definition
if pt fails steroids/medical tx
-removeds IgG source (splenic B cells) |
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Term
ITP
-pre/intra-op splenectomy mgmt |
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Definition
-IV-IG x 1 wk (prolongs plt life)
-TF PLT after ligation of splenic art if PLT < 50
-Hydrocort 100 q8
-immunizations |
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Term
Post op splenectomy
-pediatrics mgmt |
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Definition
Prophy daily augmentin x 6 months if age<10
-prevents OPSI |
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Term
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Definition
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Term
ITP
-persistent post-op TCP work up /mgmt |
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Definition
1.smear (for asplenic chagnes)
-none? consider accessory spleen (CT)
2. tx steroids/IVIG +/- reop |
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Term
Thrombotic Thrombocytopenic Purpura
-path |
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Definition
1.Primary: ADAMTS13 deficient=uncleaved vWF
-->shear apart RBC
2. Secondary (40%): CA, BMTXp, preg, others |
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Term
Thrombotic Thrombocytopenic Purpura
-MC death |
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Definition
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Term
Thrombotic Thrombocytopenic Purpura
-sxs? |
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Definition
pentad
1. VERY low PLT
2. AMS
3. renal fail
4. Fever
5. MAHA |
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Term
Thrombotic Thrombocytopenic Purpura
-Tx |
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Definition
#1 = plasmapheresis (get rid of large vWF)
2. immuno sup (roids, vincristine, cycloP0
**RARELY NEED SPLENECTOMY** |
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Term
Thrombotic Thrombocytopenic Purpura
-% primary vs 2ndary |
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Definition
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Term
Thrombotic Thrombocytopenic Purpura
survival rate |
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Definition
90% for primary
(30% for secondary, which accounts for 40% cases) |
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Term
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Definition
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Term
OSPI
-pts/factors for increased risk? |
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Definition
1.MC kids < 5 (younger the pt, higher the risk)
2. MC < 2years post op
3. MC w/non trauma
4. thalassemia has further inc risk
**highest = wiskott aldrich syndrome** |
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Term
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Definition
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Term
| OPSI prevention strategies? |
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Definition
1. delay spelenctomy until age 5 (allows Ab formation and full immunization)
2. Immunize: pneumococc, meningococc, H flu
-2 wks pre-op or 2 wks post-op if trauma
3. Q3 year booster vs pneumococc + yearly flu
4. 6 months prophy augmentin if age <5
5. EARLY and BROAD abx for suspected infxn
6. abx prophy @ dental procedures |
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Term
|
Definition
| -MC = painless, swollen neck LN |
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Term
Hodgkins Lymphoma
-characteristic cell type? |
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Definition
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Term
Hodgkins Lymphoma
-best vs worst prog subtype? |
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Definition
Lymphocyte predominant = better prog
(depleted = worse)
-MC subtype = nodular sclerosing |
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Term
| Lymphoma type and pattern of node spread? |
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Definition
Hodgkins = contiguous/orderly
NHL = diffuse nodal/extranodal (non-contig) |
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Term
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Definition
B cell (90%)
*worse prog than hodgkins* |
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Term
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Definition
fever/night sweats, weight loss (worse prog)
*constitutional sxs) |
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Term
|
Definition
1.excisional bx, 1 cm^3 min (need architecture) **NO FNA OR CORE bx**
2. BMBx
3. CT/PET or MRI (for spleen) |
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Term
|
Definition
-A = asympt (B = for B symptoms)
1 = 1 LN region
2 = 2+ LN regions (non-contig), same side of Diaphragm
3 = LN on both sides of diaphragm
4 = disseminated (1+) extra-lymphatic organs (non-splenic) |
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Term
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Definition
1. ABVD = doxorub(adriamycin), bleomycin, vinblastine, dacarbazine
2. XRT for bulky dz *high risk for XRT induced malig* |
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Term
hodgkins tx
-treatment induced CA types? |
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Definition
1.XRT+chemo --> leukemia, lung CA from
2. XRT only ==>breast
**also early CAD** |
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Term
|
Definition
1. CHOP-R (cycloP, doxorub(hydroxy), vincristine, prednisone, ritux)
2. XRT vs bulky dz
*add MTX for CNS prophy if high risk* |
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Term
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Definition
1. LN for dx
2. for tx of chylous effusion
**rare splenectomy needed**
**rarely need staging lap these days** |
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Term
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Definition
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Term
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Definition
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Term
| 4 cardinal signs of hypersplenism ? |
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Definition
1. Cytopenia
2. Compensatory BM response
3. Splenomeg
4. Correction of cytopenia after splenect |
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Term
hemolytic anemias
-list of membrane protein defect |
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Definition
1. hereditary spherocytosis
2. hereditary elliptocytosis |
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Term
hemolytic anemias
-list non membrane protein causes |
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Definition
1. pyruvate kinase def
2. warm Ab AI hemolytic anemia
3. Beta thal
4. sickle cell
5. G6PD def |
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Term
| MC congen hemolytic anemia requiring splenectomy? |
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Definition
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Term
Hereditary sphereocytosis
-PPHYS |
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Definition
| -spectrin defect-->deformed cells ==>splenic sequestration ===>splenomegaly |
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Term
Hereditary sphereocytosis
-Sx
Tx |
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Definition
-pigmented stones, anemia, J,
Tx = splnectomy and chole |
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Term
Hereditary elliptocytosis
-pphys |
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Definition
| Spectrin and Protein 4.1 deficit |
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Term
| MC congen hemolytic anemia NOT INVOLVING membrane protein, that requires splenectomy? |
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Definition
| Pyruvate kinase deficiency |
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Term
Warm AB AI hemolytic anemia
-pphys
-indications for surg? |
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Definition
-IgG vs RBC (MC AI hemolytic DZ)
-splenec if refract to roids/IVIG |
|
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Term
Beta thal
-PPHys/types
-sx |
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Definition
1. Major = both chains vs minor = 1 chain
2. persisteant Hgb F
-Major = sx = pallor, FTT, big head
-minor = asympt
**most die as teens 2/2 hemosiderosis** |
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Term
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Definition
| Hgb A replaced with Hgb S |
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Term
G6PD def
-precipitating factors? |
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Definition
|
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Term
|
Definition
RA, neutropenia, splenomeg, infxns,
*occasional splenectomy needed vs splenomeg w/neutropenia* |
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Term
| Conditions ALWAYS needing splenectomy (4) |
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Definition
1. hered sphero
2. hered ellipto
3. splenic vein thrombosis w/gstric variceal bleed
4. echinococcal cyst (MC splenic cyst) |
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Term
| conditions USUALLY needing splenectomy (5) |
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Definition
1. refract warm AB AI HA
2. refract adult ITP
3. aplenic abscess (MC strep, then staph)
4. Isolated splenic lymphoma (rare)
5. MM |
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Term
| MCC spontaneous splenic rupture |
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Definition
Mononucleosis = MC
-others = malaria, sepsis, sarcoid, luekemia, PCVera |
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Term
|
Definition
| MC after truama (splenic implants) |
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Term
Myeloid metaplasia
=meaning? |
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Definition
| spleen acting as bone marrow |
|
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Term
isolated lymphoma of SB
-mgmt |
|
Definition
1. duod: Chemo XRT
2. ileum/jej: wide en-block, then CXRT |
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Term
MC location colonic lymphoma?
mgmt |
|
Definition
cecum
-wide en block f/b CXRT |
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Term
|
Definition
|
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Term
|
Definition
|
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Term
|
Definition
surg only if stg 1 dz
-otherwise CXRT |
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Term
|
Definition
|
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Term
| splenic art vs vein relative location |
|
Definition
vein = posterior + inferior to art
*both post to spleen* |
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