SPLEEN. Professor Anwar Sheikha THE SPLEEN MAN IN THE SPLEEN LAND.
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Transcript of SPLEEN. Professor Anwar Sheikha THE SPLEEN MAN IN THE SPLEEN LAND.
SPLEEN
Professor
Anwar SheikhaAnwar SheikhaTHE
SPLEEN MANIN THE
SPLEEN LAND
FUNCTIONSOF THE
SPLEEN
IMMUNOLOGICAL
PHAGOCYTOSIS OF PARTICUALTE
MATTERS
BLOOD POOLINGREGULATION OF ERYTHROPOIESIS
EMH
FUNCTIONSOF THE
SPLEEN
Main Police &Security Force
مركزشرطةوأمنالبلد
SEWERAGEDISPOSALالمجاري
GRAVEYARDالمقبرة
POOLINGالمخزن
األحتياطEMH
FUNCTIONSOF THE
SPLEEN
مركزشرطةالمجاريوأمنالبلد
المقبرة األحتياطالمخزن
FUNCTIONSOF THE
SPLEEN
پوليسوئهمنىناوشار
زبلوئاوهرو
ورستانگ سايلواحتياط
SPLEEN
MYSTERII ORGANUM PLENUM
EXTRACTION OF MELANCHOLIC HUMOURS
GREAT LAUGHTERS HAVE GRAET SPLEENS
SPLEENGALEN’S
ORGAN OF MYSTERY
HEMATOLOGIST
SURGEON IMMUNOLOGIST
INFECTIOUSDISEASE
PHYSICIAN?
WHO
WHO SHOULD SEE A PATIENT WITH SPLENOMEGALY?
WILLAIM CRSOBY
ROMANCING THE SPLEEN HAS BEEN A PLEASURE,
BUT MARRIAGE WAS
OUT OF THE QUESTION
SPLENOMEGALY
INFECTIONSACUTE SUBCUTE CHRONIC
PARASITIC
TYPHOIDPARATYPHOID
TYPHUS
INEFCTIOUS MONO-
NUCLEOSIS
INFECTIOUSHEPATITS
BRUCELLATOXOPLASMA
SEPTICEMIA
S.B.E.
T.B.
BRUCELLA
SYPHILIS
HISTOPLASMA
CHRONIC MENINGEALSEPTICEMIA
MILDSMG
HYDATID
TRYPANOSOMA
KALA AZAR
MALARIA
MILDSMG
LATE IN REGRESSIVESPLENOMEGALY
EARLY IN PROGRESSIVESPLENOMEGALY
DISORDERS WITH OCCASIONALSPLENOMEGALY
ITP
SLE
FELTYMYELOMA
SARCOID
MEGALO-BLASTIC ANEMIA
CHRONIC IRON
DEFICIENCYANEMIA
AMYLOID
MODEARTESMG
LYMPHOMAS
PRV
CLL
ACUTELEUKEMIA
HEMO-LYTIC
ANEMIAPORTAL
HTN WITHCONGESTIVE
SMG
CML THAL.
MYELOFIBROSIS
CYSTSTUMORSGAUCHER
PARASITIC KALA AZARTSS
BILHARIZIA
MARKEDSMG
CAUSES OF SPLENOMEGALY
PATHOGENESIS OF
SPLENOMEGALY
LYMPHOMA
HYPERPLASIA
EMH
RED CELL POOLINGCONGESTIVE
INFLAMMATORY
MISCELANEOUS
STORAGE
SMG
CONGE-STIVE
Cirrhosis
NEOPLASIA
Leukemias (AL; CML; CLL) Lymphomas Metastasis
EMHThalassemiaOsteopetrosisMyelofibrosis
HA
H. S.H.E.Thal
STORAGE DISEASESGaucher; NPDHistiocytosis
Mucopolysac.
INFECTIONSBacterial Parasitic
V.L.
TSS Bilh.
TSSTROPICAL
SPLENOMEGALY SYNDROME
MALARIAL SPLENOMEGALY
TSSTROPICAL
SPLENOMEGALY SYNDROME
MALARIAL SPLENOMEGALY
TSSMAJORDIAGNSOTC
CRITERIA
Gross Splenomegaly
Immunity to Malaria
High Serum IgM
Clinical & immunological Response to Antimalarial
MINORDIAGNSOTC
CRITERIA
Hepatic Sinusoidal lymphocytosis
Normal Immune response to Antigenic Challenge
Normal PHA Response
Hypersplenism
Lymphocytic Proliferation
Occurrence in families
SYMPTOMS % SIGNS %Abdominal Swelling
65 Massive splenomegaly
100
Abdominal Pain 50 Hepatomegaly 90Cough 13 Pallor 30Weakness 12 Jaundice 20Leg Swelling 10 Hemic Murmur 5Epistaxis 5 Leg Ulcer 5Hernia 5 Hernia 2
CLINICAL FEATURES OF TSS
PATHOGENESIS OF TSS
MALARIA PARASITE
B- LYMPHOCYTE
IgM ++++
HIGH M. Wt. IMMUNE COMPLEXES
SPLENOMEGALY
HELPER T- CELLS SUPPRESSOR T
- CELL
S
x
DACIE’S SYNDROME
IDIOPATHIC NON-TROPICAL SPLENOMEGALY
GROSS SPLENO- MEGALY
HYPERSPLENISM
NO SYSTEMIC
DISEASE
DACIE’S SYNDROME
* A 45 YEAR OLD FARMER* 3 MONTHS H/O TIREDNESS & NIGHT SWEATS* O/E PALLOR & GROSS SPLENOMEGALY
Hb: 53 g/LWBC: 1,900/UlPlat. 52,000/ul
Marrow: Active
RADIONUCLIDE STUDIES:RCM: 13.8 ml/KgSplenic Red Cell Pool: 28%T50 RBC Survival: 22 daysPlasma Volume: 60 ml/Kg
SPLENECTOMY
Weight: 1570 gm
NO SPECIFIC FEATURES
17 YEARS LATER PATIENT WAS NORMAL WITH NORMAL CBC
GAUCHER’SDISEASE
I
ADULT“NON-NEUROPATHIC”
SPLENOMEGALYHYPERSPLENISM
PORTAL HTN
II
INFANTILE“NEUROPATHIC”
EARLY DEATH
III
JEUVENILE
PREDOMINANTLYNEUROPATHIC
↓β –
GLUCO-CEREBRO-
CIDASE
AshkenaziJews
FELTY’SSYNDROME
RHEUMATOIDARTHRITIS
SPLENOMEGALY10%
NEUTROPENIA1%
ANEMIA
TP
PIGMENTATION
INFECTIONS
GALL STONESLEG ULCERS
PUL. HTN
SERIOUS BACTERIAL INFECTION IS RELATIVELY UNCOMMON
SPLENECTOMY DOES NOT ALTER COURSE OF FELTY’S
HYPERSPLENISM
HYPER-SPLENISM
HYPERCELLUALR OR
NORMOCELLULARBONE MARROW
CYTOPENIA
ANEMIANEUTROPENIA
THROMBO-CYTOPENIA
SPLENOMEGALY
CORRECTION O FTHE CYTOPENIA
AFTER SPLENECTOMY
GRAVEYARD ABBATOIRE
HYPER-SPLENISM
HYPERCELLUALR OR
NORMOCELLULARBONE MARROW
CYTOPENIA
ANEMIANEUTROPENIA
THROMBO-CYTOPENIA
SPLENOMEGALY
CORRECTION O FTHE CYTOPENIA
AFTER SPLENECTOMY
گورستان گردىشههيدان
HYPER-SPLENISM
HYPERCELLUALR OR
NORMOCELLULARBONE MARROW
CYTOPENIA
ANEMIANEUTROPENIA
THROMBO-CYTOPENIA
SPLENOMEGALY
CORRECTION O FTHE CYTOPENIA
AFTER SPLENECTOMY
مقبرة جزرةم
HYPO-SPLENISM
GICELIAC
D. HERPETIFORMISULCERATIVE COLITIS
CIRRHOSIST. SPRUE
MISCELANEOUS
IRRADIATONAMYLOIDOSISSARCOIDOSIS
GvHD
HEMATOLOGICAL
SICKLEE.T.
AUTOIMMUNE
SLE
RACAH
CTD
CONGENITALSURGICAL“SPLENECTOMY”
USUALLY SOMETIMES OCCASIONALLY
H.S. AIHA MF
CHRONIC ITP ACUTE ITP CLL
HYPERSPLENISM LYMPHOMAS
HCL H.E.
THAL. MAJOR
FELTY’S
INDICATIONS FOR SPLENECTOMY
INDICATIONSFOR
SPLENECTOMY
SURGICAL
DIAGNSOTICLYMPHOMA
STAGING
THERAPEUTICITP
HCL
MFCML H.A.
HS
HE
AIHAThal. & HbSS
CDA
HYPER-PLENISM
Pitted Red Cells
OPSI
Overwhelming Post-Splenectomy Infection
OPSIORGANISMS
VIRUSUSCMCHZVEBVHIV
PROTOZOA
MALARIA
BABESIAENCAPSULATEDBACTERIA
Strep. PneumonaieH. influenza tyoe b
N. meningitides
OPSIRATE
TRAUMA 1.5%
HEMATOLOGICAL 3.5%
PORTAL HTN 8.2%
HODGKIN 10%
SICKLE 15%
THALASSEMIA 25%
OPSIRATE
AGE (YEARS) OPSI
1 - 16 9-20%
<1
>1
50%
2.8%
<5
>5
8.1%
3.3%
<1
>1
21%
3.5%H.S.
Avoid splenectomy when possible
Delay splenectomy until patient is >5 yr
Immunize before splenectomy with pneumococcal vaccine, meningococcal vaccine & Hib vaccine
Daily prophylactic antibiotic administration (penicillin or amoxicillin orally or erythromycin for allergic
patients) for all high risk patients of development of sepsis (i.e., younger patients, underlying disease)
Aggressive education of patient and family to ensure rapid medical attention & Antibiotics
Partial rather than total splenectomy
Obtain MedicAlert or equivalent warning system
Prevention of OPSI
Antibiotic Doses for Prophylaxis for Patients with Asplenia/Hyposplenia
Age Pencillin V Amoxicillin Erythromycin (EES)a
2 mo to 3 yr 125 mg p.o. b.i.d. 125 mg p.o. b.i.d. 125 mg p.o. q.i.d.
3 yr and older 250 mg p.o. b.i.d. 250 mg p.o. b.i.d. 250 mg p.o. q.i.d.
Emergency Management of Febrile Asplenic Patients
Temperature below 38.5ºC
Examine patient (vital signs, localizing findings, especially any indication of meningitis).
Obtain blood culture and other cultures as indicated.
Administer stat intravenous or intramuscular antibiotics (ceftriaxone, ampicillin, or equivalent coverage).
Begin oral antibiotics to cover encapsulated organisms (choice depends on local epidemiology and whether the patient is receiving prophylactic antibiotics).
Temperature above 38.5ºCExamine patient (vital signs, localizing findings, especially any indication of meningitis).
Obtain blood cultures, CBC & other cultures as indicated.
Administer stat i.v. antibiotics (ceftriaxone, ampicillin, or equivalent coverage).
Maintain intravenous hydration and observe patient for at least 6-12 hr in medical facility.
Consider hospital admission, depending on local circumstances, ability to monitor patient, and proximity to medical facility. Discharge requires a responsible adult immediately available to bring patient back in event of change in clinical stat
A CASE STUDY
SHAYKHA MOH’D ASIRI
AGE: 70 YEARSSEX: FEMALENATIONALITY: SAUDIHOSP. #: 014450
MARKEDSPLENO-MEGALY
MILDPAN-CYTO-PENIA
? MYELOFIBROSIS
HISTORY:Admitted Muharam 1409 to KCH
3/12 H/O Generalized WeaknessInability to take full mealsMarked Abdominal Distension
NOFever Pruritis CoughSweating Bone Pain SputumWt. Loss Bleeding Chest Pain
No Lymphadenomegaly
PAST HISTORY
SOCIAL HISTORY NIL OF NOTEFAMILY HISTORY
PRESUMPTIVE DIAGNOSIS IN THE REFERRING HOSPITAL MYELOFIBROSIS
O/ESmall, old, Frail & Cachectic LadyMild PallorNo Jaundice
WEIGHT
44Kg
Abdomen: Huge Spleen occupying almost all of the AbdomenChest, CVS, CNS, etc: Unremarkable
INVESTIGATIONS
Hb: 101 g/LWBC: 5,400 /uLPlatelets: 90,000 /uLNeutrophils: 2,000 /uL
MCV: 84 FlRetic. 6%Smear: OKCoomb’s: NegativeClotting: NormalMalaria: Negative
BLOOD BIOCHEMISTRY: NORMAL
TOXOPLASMABRUCELLABILHARIZIA
LEISHMANIA
SEROLOGY NEGATIVE
SERUM PROTEIN ELECTROPHORESIS: NORMALIMMUNOGLOBULIN QUANTITATION: NORMALANF: NEGATIVE
BONE MARROW: NOT DIFFICLUT
ASPIARATION: ACTIVE; LYMPHOID CELLS <15%
BIOPSY: ACIVE & HYPERCELLULARNO EVIDENCE OF MYELOFIBROSISLYMPHOID CELLS:NOT INCREASED
RADIOLOGYChest X-Ray: NormalAbdominal Ultrasound: Huge Spleen
No Focal massesKidneys, Liver & GB: Normal
CT SCAN:NO MEDIASTINAL WIDENINGMARKED SPLENOMEGALY
NO ABDOMINAL LYMPHADENOMEGALY
DIAGNOSIS
??
MYELOFIBROSISSMEARNORMAL
RETICULINNORMALMARROW
EASY
SUMMARY:AN OLD CACHECTIC LADYMASSIVE SPLENOMEGALYMILD HYPERSPLENISM
DIAGNOSTIC & THERAPEUTICSPLENECTOMY
SPLEEN: GROSLLY ENLRAGEDWEIGHT: 2400 GRAMSFINELY NODULAR
MICROSCOPY: NON-HODGKIN’S LYMPHOMAFOLLICULAR SMALL CELLNODULAR
PRIMARY LYMPHOMA OF THE SPLEEN
POST-OPERATIVEMANAGEMENT
?
CHEMOTHERAPY
?
WAIT & WATCH
FOUR YEARS LATER
SHE PUT ON 10 KILOGRAMSNO EVIDENCE OF LYMPHOMA
CURED
TEN YEARS LATER
SHOULD I HAVE USED CHEMOTHERAPY?
THANK YOU
