SPLENOMEGALY

DR : A.A.MOKHTAR

2016
INTRODUCTION
NORMAL SPLEEN
 Normal size – 12 cm length , 7 cm width
(radionuclide scan)
-13cm craniocaudal diamtr (USG)

 weight- < 250gm

 Located along- 9th, 10th,11th ribs mid-axillary

 Spleen should be twice the size to be PALPABLE

 Palpable spleens are not always ABNORMAL

 3% normal population has palpable spleen
inch thick 1
inches broad 3
inches long 5
ounces weight 7
underlies 9-11 ribs
FUNCTIONS
 Quality control over RBC pitting

 Synthesis of antibodies

 Removal of antibody coated bacteria & RBC

The white pulp of the spleen
contains macrophages, B
lymphocytes, and T lymphocytes,
participates in the recognition of
microorganisms and foreign
proteins, and is involved in the
primary immune response
The splenic red pulp occupies
more than half the volume of
the spleen and is the site where
senescent red cells are
identified and destroyed and
red blood cell inclusions are
removed by a process known
as pitting
Spleen is the largest lymphoid organ organ and serves following
functions –
1. Red pulp: Removal of senescent and defective RBCs
(mechanism: hypoxia, low pH and low glucose)
2. White pulp: Synthesis of antibodies
3. Removal of antibody-coated bacteria and anti-body coated blood cells from circulation
4. Extramedullary hematopoiesis
5. Blood pooling

An increase in these normal functions may

result in splenomegaly
WHAT HAPPENS IF WE REMOVE
SPLEEN?

These patients are more susceptible to

bacterial sepsis,especially with
encapsulated organisms.
WHAT HAPPENS IF WE REMOVE
SPLEEN?
 Without spleen, different abnormal red
cells will be present in blood circulation:
 senescent red cells
 red cells with nuclear
remnants(Howell-jolly bodies)
 insoluble globin precipitates(Heinz
bodies)
EXAMINATION OF SPLEEN
EXAMINATION OF SPLEEN
 Inspection
 Palpation - bimanual method,hooking maneuver

 Percussion – nixon method

- rt.lateral ducubitus, > 8cm
- castells method
supine,lower ICS ,full exp & insp
splenomegaly=dullness
- traubes sign
supine,6th rib,costal margin,
anterior axill. line
splenomegaly= dullness
BIMANUAL PALPATION
DIFFERENCES
spleen kidney

 Sharp edge  Round edge

 Notch –med border  No notch

 Cross midline  Not cross midline

 Moves with respiration  Not moves with resp.

 Cannot get above it

 Can get above it

TRAUBE’S SPACE
Traube space is defined by the sixth rib
superiorly, the left anterior axillary
line laterally, and the costal margin
inferiorly. Castell spot is located at the
junction of the lowest intercostal space
and the left anterior axillary line.
Nixon Method to Detect Splenomegaly
Nixon method of percussion requires that the patient be placed in the right
lateral decubitus position.
Percussion is started at the midpoint of the left
costal margin and proceeds perpendicularly.
Splenomegaly is diagnosed when the dullness is present more than 8 cm above
the costal margin.
 MECHANISMS OF SPLENOMEGALY

1. Work hypertrophy
Reticuloendothelial system hyperplasia (Removal of defective erythrocytes)
Immune hyperplasia (Response to infection or disordered immunoregulation)
Extramedullary hematopoiesis (Bone marrow disorder)

2. Infiltration
Acellular deposition: Metabolic disorders
Cellular deposition: Neoplasia

3. Passive congestion
 Grades OF SPLENOMEGALY
HYPERSLENISM
 Splenomegaly

 Pancytopenia

 Presence of hypercellular marrow

 Reversal with splenectomy

SYMPTOMS OF SPLENOMEGALY
 Pain

 Early satiety

 Feeling of heaviness in LUQ

CAUSES OF SPLENOMEGALY
 Infective

 Hyperplastic

 Congestive

 Infiltration
CONGESTIVE
 Intra hepatic obst.portal hypertension
- cirrhosis,biliary cirrhosis,hemochromatosis
- primary sclerosing cholangitis
 Extra-hepatic portal hypertension

- venous malf,thrombosis,stenosis
- ext.occlusion of portal,splenic vein
 Chronic passive congestion of cardiac origin
HYPERPLASTIC
 Extramedullary hemopoeisis- myeloprolif.d/s
- marrow damage
- marrow infiltration

 Reticulo endothelial hyperplasia –(abn.RBC)

- sickle cell d/s,spherocytosis,Hbnopathies,
thalassemia major,PNH
INFILTRATIVE
 Malignant infiltration- CML,lymphoblastic
- lymhomas, MPD,
- angiosarcoma,tumors
- metastasis (melanoma)
 benign -

- storage d/s –Gaucher’s,Neiman-pick

- amyloidosis
- hurler’s syndrome,MPS
- cysts,fibromas,hemangiomas,hamartomas
-Eosnophilic granulomas
DISORDERED IMMUNOREGULATION
 Felty’s syndrome- RA+ splenomegaly+leucopenia

 Systemic lupus erythromatosis

 Collagen vascular diseases

 Sarcoidosis

 Immune thrombocytopenia
MASSIVE SPLENOMEGALY (>8CM
>1000GM)
 Myeloproliferative disorder
 Chronic malaria,kala-azar (trop. Splenomegaly)

 Storage disorders

 Thalassemia major

 Sarcoidosis

 Hairy cell leukemia

 Gaucher disease

 Diffuse splenic hemangiomatosis

 Schistosomiasis,
MODERATE SPLENOMEGALY(4-8CM)
 Cirrhosis
 Lymphomas‘

 Amyloid

 Splenic abscess,infarct

 Hemolytic anemias

 IMN
MILD SPLENOMEGALY (1-3CM)
 Acute infective conditons
 Acute malaria,tyhoid,kala-azar,septicemias
DIAGNOSTIC APPROACH
STEP-WISE APPROACH TO
SPLENOMEGALY
 History
 Physical examination

 Laboratory testings

 Imaging

 Specialised testing
 HISTORY
 1. Suggestive of splenomegaly:

 Pain and a heavy sensation in LUQ radiating to back

 Radiation to left shoulder tip in splenic infarction
 Early satiety in massive splenomegaly
2. SUGGESTIVE OF ASSOCIATED
DISEASE:

 Syst symptoms i.e. Fever, night sweats, or weight loss (Neoplastic,

SBE and other infections)
 Bone pain (AML, Sickle cell disease, Gaucher’s disease)

 Fatigue, dyspnea, bruising and/or petechiae (Hemolytic)

 Joint pains (RA, SLE)

 Pruritus (Hodgkin lymphoma, Polycythemia)

 Epigastric or generalized abdominal pain (Splenic vein

thrombosis eg. Pancreatitis)

 Cough and dyspnea (Sarcoidosis)

 History of alcoholism, liver disease (Liver cirrhosis)

 History of Pancreatitis (Splenic vein thrombosis)

 Personal or family history of hemoglobinopathy, lysosomal

storage disorder, rheumatoid arthritis

 History of neonatal umbilical vein sepsis (Portal vein

thrombosis)
 Recent infections including malaria

 History of recent dental work or blood transfusions (SBE)

 Recent abdominal trauma

B) PHYSICAL EXAMINATION:
LOCAL EXAM :
1. Inspection: Fullness in LUQ that descends on inspiration
(massive splenomegaly)
2. Palpation: Spleen is not normally palpable (palpable
when 2-3 times enlarged).
Enlargement takes place in a superior and posterior
direction before it becomes palpable subcostally. A
palpable spleen must be reported in following points:
 Degree of enlargement: Measured below from the left
costal margin along the splenic axis in centimeters or
number Of fingers
 Splenic notch: Felt on its lower medial border

 Margin: Usually sharp

 Consistency: Soft, firm or hard
 Tenderness: Tender or non-tender

 Surface: Smooth or irregular

 Movement with respiration: Always moves downwards

and medially with respiration
 Fingers insinuation: cannot get between spleen and ribs

 Palpable splenic rub: Present or not.

3. Auscultation: Venous hum or a friction rub may be heard

4. Percussion: Palpation is confirmed by dullness as spleen
is dull to percussion
A PALPABLE SPLEEN IS DISTINGUISHED FROM
PALPABLE LEFT KIDNEY MASS BY:

 Not bimanually palpable and not ballotable

 Upper border cannot be felt

 Notch on lower medial border

 Fingers cannot get between spleen and ribs

 Dull on percussion
OTHER RELEVANT FINDINGS IN
PHYSICAL EXAMINATION:
Skin:

 Pallor: Chronic malaria, Chronic kala-azar, Leukemia,

Lymphomas, Cirrhosis, Hemolytic anemia, Hypersplenism
 Icterus: Hemolysis (Hemolytic anemias, Acute malaria,

 Lymphoma), Budd-Chiari syndrome (Hepatic vein obstruction),

Chronic liver disease

 Periorbital purpura: Amyloidosis

 Plethora: Polycythemia vera

 Skin infiltration and masses: AML or ALL

 Butter fly rash: SLE

 Janeway lesion: SBE

 Erythema nodosum, lupus pernio: Sarcoidosis

 Spider naevi and palmar erythema: Portal HTN due to chronic

liver disease
 Hemorrhagic spots: Acute leukemia, SBE, SLE, ITP, Felty’s

syndrome, Blast crisis of CML or CLL

B. LYMPHADENOPATHY:

 Autoimmune disorders: RA, Felty’s syndrome, SLE,

Sarcoidosis
 Infection: Infectious Mononucleosis, AIDS,
Toxoplasmosis, CMV,
 Disseminated TB

 Neoplasm: Lymphomas and Leukemias

C. FEVER
 Infections:
 Malaria, Kalaazar,

 Enteric

 fever, SBE,

 Miliary TB,

 Acute viral

 hepatitis

 Neoplasm:

 Acute

 leukemias, CML,

 Lymphoma

 Collagen vascular diseases

D. EYES AND ENT

 Fundoscopy: Roth spots (SBE), Choroidal tubercle

(Miliary TB)
 Pharyngitis: EBV infection

 Macroglossia, Jugula vein distension or Periorbital

edema: Amyloidosis
 Mongoloid facies: Thalassemia
E. CARDIAC EXAMINATION:

 New or changing murmurs: SBE

F. EXTREMITIES:

 Digital ischemia/gangrene or thrombosis: Essential

thrombocytosis
 Joint deformities: RA, Felty’s syndrome, SLE

 Lower extremity edema: Amyloidosis

G. ABNORMAL NEUROLOGICAL EXAMINATION:

 Essential thrombocytosis
 Non-Hodgkin’s lymphoma (NHL)
DIFFERENTIAL DIAGNOSIS OF A MASS IN THE
LEFT HYPOCHONDRIUM:

1. Enlarged left kidney

2. Enlarged left lobe of liver
3. Carcinoma of stomach
4. Carcinoma of splenic flexure of colon
5. Omental mass (TB or malignancy)
6. Malignancy of tail of pancreas
7. Ovarian tumor in females
LAB INVESTIGATIONS
 CBC
 Blood smear

 Retic count

 Blood C/S

 Serology (fungal,viral,parasitic)

 LFT

 Hb electropheresis/ coombs test

 Coag.profile

 Amylase/lipase

 AMA, Anti CCP,RA factor

 Bone marrow analysis

 IMAGING
 USG- sensitive & specific non-invasive
 CT scan – etiology of splenomegaly

- liver size,heterogenecity
- splenic mets, abscess,calcf.,cysts
- retro peritoneal LN
- craniocaudal ln > 10 cm
 Liver- spleen colloid scan- (RBC –Cr51,Tc99)

- hepatic steatosis,SOL,splenic functions

- PHT,colloid shift +
 MRI/ Doppler usg- portal/splenic vein thrombosis

- cavernomas
IMAGING
 MRI scan- liver hemangiomas
hemochromatosis
erlenmeyer flask sign(Gaucher)
 PET scan - Dx & staging of lymphomas

- determine metabolic cells in spleen

SPECIALISED TESTING
 Abd.fat pad aspiration
 JAK-2 mutation

 Gene testing(bcr-abl ,C282Y)

 Enzyme testing

 Lymph node biopsy

 FNAB spleen

 Splenectomy

 Lung or skin biopsy

 Liver biopsy
SPECIAL SITUATIONS ASSOCIATED
WITH SPLENOMEGALY
 Fever- typhoid,malaria,kalaazar, infect.endocarditis,
leukemia,lymphoma
 Tender spleen- rupture,abscess,infarct

 a/c illness+ anemia- AIHA,leukemia

 Fever + LN- IMN,leukemia,lymhomas,SLE,sarcoid

 Anemia- hemolytic anemia,hemoglobinopathies

 Jaundice – cirrhosis,hemolytic anemia

 Pulsatile spleen- aneurysm

 High ESR- connective tissue disorder

 Leukopenia- felty’s syndrome,septicemia

TROPICAL SPLENOMEGALY (HMS)
 Massive splenomegaly
 Endemic areas of malaria,kala-azar

 IgM antibodies +

 No parasite in blood

 Lymhocytic infiltration of splenic sinusoids

 Long term anti-malarials

SUMMARY
 Splenomegaly – major physical finding

 Step wise approach- history,physical exam

 Look for associated features

 Lab investigation & Imaging

 Search for etiology & treat