SPLENO MEGALY –INTEGRATED APPROCH TO DIAGNOSIS
Basics
Causes of Splenomegaly
I. Primarily hematological
II. Non hematological
I. Primarily hematological:
1. Neoplastic
2. non Neoplastic
I.1.
Neoplastic-
a) Leukemias
· Chronic lymphocytic
Including prolymphocytic and hairy cell
· Chronic granulocytosis
· Acute lymphoblastic
· Acute myeloblastic(less common)
b).Polycythemia rubra vera
C). Lymphoma
d). myelodysplastic syndrome
e)Histiocytosis
I.2.
Non Neoplastic
Hemolyic anemias (mainly)
Megaloblastic,iron deficiency anemia(minimal enlargement)
Auto immune thrombocytopenia(rare)
II.non Hematological
1. Infective
( mechanism-accumulation of inflammatory cells)
(splenic filtration of blood born pathogens may lead to indolent abscesses ;as they enlarge,spleen also enlarges)
a.acute and subacute infections
.infectious mononucleosis
endocarditis
severe pyogenic infections
a. Chronic
TB,Syphilis.brucellosis
b. TropicalSplenomegaly
c. Malaria,leishmaniasis,trypanasomiasis
The term tropical splenomegaly is generally reserved for chronic splenic enlargement in
patients from malarial areas but its occurance bears no relation to the apparent severity of
infection.;malarial parasite not routinely seen in peripheral smear
2
.Congestive
( Mechanism-Normal spleen helps to regulate portal blood flow;
(Disordered splenic blood flow causes splenic congestion)
2a. Intrahepatic obstructive portal hypertension
Portal cirrhosis,biliary cirrhosis,post necrotic scarring
Hepato lenticular degeneration
Hemochromatosis
Hepatic venous occlusion
Sarcoidosis
2b. Extra hepatic obstructive portal hypertension
Venous malformations,thrombosis,,atresia,stenosis,aneurysmal formation
Extensive occlusion of i) portal vein ii) splenic vein
2c.Chronic passive congestion of cardiac origin
3
.Infiltrative
Malignant infiltration
Leukemia- CML,Lymphoblastic
Lymphoma-hodgkins,Non hodgkins
Myelo proliferative syndrome
Angio sarcoma
Primary splenic tumor
Benign
Extra medullary hematopoiesis
Storage diseases
Gauchers
Niemann picks disease
( Mechanism-Metobolite laden macrophages)
Amyloidosis
Hurlers syndrome
Eosinophilic granulomas
Mucopoly sacchridosis
4.a. Conditions with Abnormal Erythrocytes
Mechanism-sequestration and removal of abnormal blood cells)
4a. Spherocytosis,
Sickle cell disease,(before auto splenectomy occurs)
Ovalocytosis
Thalassemia
4b.Hyperplastic splenomegaly due to work hypertrophy
Extramedullary hematopoiesis-Myeloproliferative disease
5.Connective tissue disease
( Mechanism-splenomegaly due to disordered immune regulation)
.Rheumatoid arthritis-felty’s syndrome.
(Componants:arthritis,splenomegaly and leucopenia)
.Systemic lupus erythematosis
6. Miscellaneous
sarcoidosis
Cysts and hemorrhage
Aneurysm of splenic artery
Metastatic tumors primary hamartoma
Idiopathic
Thyrotoxicosis
Iron deficiency anemia
Degree of splenic enlargement and diagnosis
Note:
A palpable spleen is not always an enlarged spleen(visceroptosis or displacement)
‘Normal size-12cm length7cm width’;
Located along long axis of Left 9th,10th and 11th rib
in -mid axillary line
Spleen should be twice the normal size to become palpable.
I.Causes of massive splenomegaly (beyond umbilicus)
In very large spleen -lower splenic pole may extend into pelvis and cross abdominal midline.
1. Myeloproliferative disorder
(proliferation of stem cells)
Chronic myeloid leukemia
Polycythemia
Thrombocytosis
2. Chronic malaria,kalaAzar(Tropical splenomegaly)
Plus uncommon causes like
3. Storage diseases
Gauchers,NemannPicks disease
4.Thalassemia major
5.Some cases of portal hypertension
6.Sarcoidosis
7.Congenital syphilis
8.Hairy cell leukemia
Disease excluded by massive spleen are Acute infective diseases
II.SMALL SPLEEN (just palpable)
Mostly in acute infective conditions
Acute malaria,typhoid,kalaazar,septicemia
III.Moderate spleen ( between costal margin and umbilicus)
Cirrhosis
Lymphoma
Hemolytic anemias
Amyloid
Splenic abscess,infarct
Hepatitis
Infectious mononucleosis
Hypersplenism; Definition:
Enlarged spleen with increase in normal destruction of blood cells .
Characterized by
1.Splenomegaly
2.. Pancytopenia (Involving all 3 blood elements or one or two)
3.Presense of (normal) or hyper active marrow usually.
4.. Reversible by splenectomy.
Investigations
· Lab studies to do: as appropriate:
CBC, blood smear for cellular morphology and parasites, culture of blood and other sites,
,serology(fungal,viral,HIV,Parasitic,TPI)
Mononucleosis screen,
PPD,
· BM aspiration and biopsy),lymph node biopsy if significant lymph adenopathy is associated.
· Imaging Studies
Ultra sound Abdomen
CT scan/MRI of Abdomen
CXR if appropriate
Image: Bonemarrow aspiration and biopsy
Splenomegaly image
Spleen is situated along long axis of9th,,10th
and 11 th ribs left side.
Organs in relation:Diaphragm,Stomach,pancreas,
colon,omentum,Left adrenal
andkidney.ovar infemale
Bloodsupply
splenic arteryfromceliac trunk
Venousdrainage-splenicvein intoportal
Lymph-celiac,para aorticnodes
Nerve–sympatheticfrom celiac plexus
Function of red pulp:Destruction of
senile and abnormalRBCS
White pulp;contains small nodules rich
in lymphoid cells
Called “malphigean nodules”-
Functions immunosurvelience
Advanced info-For the benefit of Postgraduates
I.Splenomegaly with Acute illness
1.Rupture
Splenic infarct
Acute infective conditions
2.Splenomegaly with fever
Typhoid,malaria,kala azar,military tuberculosis,
Infective endocarditis,leukemia,lymphoma
3.Highly tender spleen
Rupture with sub capsular hematoma
Splenic abscess
Splenic infarct
(sickling of RBC in splenic artery, embolism-mural,endocardial,left atrial myxoma)
4..Splenomegaly +acute illness+anemia
Autoimmune hemolytic anemia
Acute leukemia
Myeloproliferative disease
5.Splenomegaly+fever+Lymphadenopathy
(with or without arthralgia)
Infectious mononucleosis
Hodgkins/non Hodgkins/Leukemia
(Acute lymphobastic or myeloblastic leukemia)
Systemic Lupus erythematosis
Serum sickness
Sarcoid
Learning point :
Normal palpapable inguinal nodes-0.5 -2ccm
Investigate-new nodes 1cm :not nodes from antecedent illness
Consider pt,s age, physicalcharacteristic of nodes,site,and associated clinical setting
Also Evaluate:
Complete history,clinical exam,presence and extent of adenopathy,Systemic/localizing symptoms/signs;acute or chronic illness ;any h/o risk factors for HIV
Inference:
Regional adenopathy indicates infection or malignancy
Lab studies to do: as appropriate:
CBC,culture of blood and other sites,CXR.PPD,serology(fungal,viral,HIV,Parasitic,TPI)
Mononucleosis screen,blood smear for cellular morphology and parasites,BM aspiration and biopsy)
6.Rupture of spleen
Trauma
Infective conditions
-malaria.typhoid,infectious mononucleosis
7.Splenomegaly with anemia
Pernecious anemia
Hemolytic anemia
Hemoglobinopathies
8.Splenomegaly with suffused conjunctiva:
polycythemia
9.Splenomegaly with jaundice
cirrhosis
hemolytic anemia
10.Pulsatile spleen
Splenic aneurysm
11.Spleno megaly with high ESR
Connective tissue disorders
12.Splenomegaly with Leukopenia
Felty,s syndrome ( Rheumatoid arthritis+splenomegaly +leucopenia)
Septicemia
13.Bantis syndrome
Congestive Splenomegaly with hypersplesim occurring in cirrhosis and portal hypertension
14. Tropical splenomegaly
Massive Splenomegaly occurring in people of endemic area for malaria/kalaazar
But no parasite is demonstrable in blood.
IgM antibodies against malaria are detected in blood.
Cause of splenomegaly is obscure.;lymphocytic infiltration of splenic /liver sinosoids present.
This condition is rare before the age 8.
Synonym-HMS-Hyper reactive malarial Syndrome
It is a diagnosis of exclusion.Long term anti malarial therapy helps.
15.Myelo proliferative syndrome
Consists of
CML
Myeloid myelo fibrosis
Polycythemia
thrombocytosis
Mechanism of splenic enlargement
1.Reactive hyperpkasia in infection and inflammation2.Proliferation of lymphoma cells
3.infiltration by other Neoplastic cells.
4.Extramedullary hemopoiesis
5.Proliferation of macrophages in response to increased destruction of blood cells.
6. Vascular congestion
Point to note in history
1. .age,occupation,2. .Race-(congenital hemolytic disordes like thalassemia
3. .Recent infections including malaria
4. Fever,weight loss,sweating (lymphoma,infections)
5. Fever or rigors- infective endocarditis
6. pain in splenic area and or shoulder pain
7. Pruritis(lymphoma)
8. Abnormal bleeding/bruising
9. Joint pains(Rheumatoid arthritis,SLE)
10. .h/o alcoholism-Portal hypertension secondary to cirrhosis
11. ..h/o alcoholism-Portal hypertension secondary to cirrhosis
12. ..h/o alcoholism-Portal hypertension secondary to cirrhosis
13. .h/o trauma-splenic hematoma
14. h/oNeo natal umbilical sepsis(portal vein thrombosis).
15. Residence and travel abroad
16. high risk sexual behavior(AIDS
17. Drugs
Point to note in clinical examination
1. Size of spleen (measure in cm below the costal margin)note-consistency,tenderness,audiable rub
2. Hepatomegaly size,consistency
3. Lymphadenopathy
4. Fever
5. Bruising
6. Oral and other superficial sepsis
7. Stigmata of liver disease
8. Stigmata of rheumatoid/SLE
9. Splinter hemorrhage,fundal hemorrhage
10. Cardiac murmur
11. Other signs of systemic diseases
12. 12.Examination of post nasal space in cervical lymh adenopathy.
Examination of lymph nodes
1. Size,2. consistency,Firm in infections/hard in carcinoma/fluctuant in cold abscess,rubbery in
lymphoma,matted in tuberculosis
3. Mobility and attachment to skin/sub cutaneous tissue
4. 4. Superficial inflammation or ulceration
5. Sinus formation
6. Tenderness and distribution
Three methods of palpation of spleen
1.Bimanual2. ballotment3.palpation from above
Clinically differentials for swelling in left hypochondrial region
1.Enlarged left kidney,2.enlarged left lobe of liver,3.carcinoma of stomach,4.Omental mass,5.Adrenal tumor,6.pancreatic tumor, 7.Ca.Coln, 8.Big ovarian tumor in female
Investigation in cases with lymphadenopathy/splenomegaly
CBC,blood smear for cellular morphology and parasites,
look for evidence of hematological malignancy
look for pancytopenia
culture of blood and other fluids/throat swab
serology(fungal,viral,HIV,Parasitic,TPI)
Mononucleosis screening
Tuberculin test,CXR(TB,Lymphoma,sarcoid)
Lymph node aspiration cytology if malignancy suspected
If unsuccessful-lymph node biopsy
i} process tissue for histological exam
ii) immunological studies of fresh tissue
iii) culture including TB
frozen for lymphocyte studies and staining for other cell types.
Lymph nodes of ‘uncertain significance’
Non invasive tests as above
Observe the progress for few weeks
If does not resolve –biopsy
If nodes of unresolved histology: with hyperplasia or non diagnostic granuloma,
Observe for a short period and re-biopsy
Subsequent biopsy may show progression to lymphoma.
Ultra sonography:
Non invasive,specific and highly sensitive -for evaluation of splenic size.
CT SCANNING
Un enhanced CT image:
Splenic attenuation is similar to that of liver
Size of enlarged spleen in cranio caudal length>10 cm
Preoperative Splenic volume can be measured by CT
Peri hilar lymph nodes detectable by CT+accessory spleen,perisplenitis,splenic abscess
CT is study of choice to diagnose inflammatory changes
But CT cannot distinguish benign from malignant lesion
CT can also detect mass lesions, cyst,infarct,calcifications
Spleno portography
Makes out preoperative portal vein patency,distribution of collaterals in shunt operations
Can identify cause of idiopathic splenomegaly in children
Angiography:
To differentiate cyst from other splenic tumors
Spleen liver colloid scan :
Non invasive scanning test
Can evaluate spleen size,and spleen weight before splenectomy
! ’+!
Splectomy and splenic biopsy can be performed in specialized institutions
But severe bleeding is a limiting complication
Histological findings
Large Foamy cells –in NeimannPicks disease
Large hyaline masses in Amyloid
Reactive splenic vasculitis- rare complication of typhoid fever
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