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