The document provides information on evaluating and diagnosing splenomegaly (enlarged spleen). It begins by describing the normal functions and size of the spleen. Methods for examining the spleen such as inspection, percussion, and palpation are outlined. Common causes of splenomegaly are then discussed, including infectious, congestive, infiltrative, and hematologic etiologies. A step-wise approach to evaluating splenomegaly involving history, exam, labs, imaging and specialized testing is presented. Specific situations associated with splenomegaly and issues regarding splenectomy are also covered.

1. approach to
splenomegaly
Chirantan Mandal
SR Medicine
AIIMS Kalyani

2. normal physiologic duty of spleen
• Maintenance of quality control over RBC in the red pulp
by removal of senescent and defective RBCs
• Synthesis of antibodies in the white pulp
• removal of antibody-coated bacteria and antibody-coated blood cells

3. • normal spleen weighs <250 g
• maximum cephalocaudal diameter of 13 cm by ultrasonography
maximum length of 12 cm & width 7 cm by radionuclide scan
• Usually not palpable
• Located along- 9th, 10th,11th ribs mid-axillary line
• long axis is lying along the direction of the left 10th rib
• Spleen should be twice the size to be PALPABLE
• Palpable spleens are not always ABNORMAL
• 3% normal population has palpable spleen
Normal Spleen

4. Examination of Spleen
Inspection
may reveal fullness in the LUQ that
descends on inspiration usually in
massive splenomegaly

5. Percussion of Traube’s space
• 6th rib superiorly
• left midaxillary line laterally
• left costal margin inferiorly.
• percuss from medial to lateral,
• normally resonant sound.
• dull percussion note in splenomegaly

6. Castell’s method
patient supine
percuss in lowest ICS 8th/9th
along anterior axillary line
resonant note if spleen is
normal
Dull if splenomegaly.

7. Nixon’s method of
percussion of spleen
begin Percussion at lower level of
pulmonary resonance in
mid axillary line &
Proceed perpendicular along
towards mid point of
anterior costal margin
upper border of dullness is 6/8 cm above
costal margin.
Dullness above >8 cm indicate splenic
enlargement

8. Palpation
Middleton maneuver can be accomplished by bimanual palpation,
ballotment & palpation from above

9. MECHANISM OF SPENOMEGALY
• Reactive Reticulo-endothelial hyperplasia
• Lymphoid hyperplasia
• Proliferation of lymphoma cells
• Infiltration by abnormal cells
• Extramedullary hemopoeisis
• Proliferation of macrophages due to RBC destruction
• Vascular congestion

10. CAUSES OF SPLENOMEGALY
• Infective
• Hyperplastic
• Congestive
• Infiltration
• Reticuloendothelial system hyperplasia

11. Infective Causes (Immune hyperplasia)
Bacterial infection
• Subacute bacterial endocarditis
• Tuberculosis
• Splenic abscess (Enterobacter,Staph Strepto)
• Bacterial septicemia
• Brucellosis
• Tularemia
• Melioidosis
• Listeriosis
• Plague
• Secondary syphilis
• Relapsing fever
• Psittacosis
• Ehrlichiosis
• Rickettsial diseases (scrub typhus, RMSF)
Viral infection
• HIV infection/chronic
• Acute mononucleosis
• Cytomegalovirus

12. Fungal infection
• Blastomycosis
• Histoplasmosis
• Hepatosplenic candidiasis
Parasitic infection
• Malaria
• Kala-Azar
• Leishmaniasis
• Schistosomiasis
• Babesiosis
• Coccidioidomycosis
• Paracoccidioidomycosis
• Trypanosomiasis (cruzi, brucei)
• Toxoplasmosis x. Echinococcosis
• Cysticercosis

13. Congestive
(Enlargement Due to Abnormal Splenic or Portal Blood Flow)
• Cirrhosis with portal hypertension
• Right-sided congestive heart failure
• Budd-Chiari syndrome (IVC & hepatic vein thrombosis)
• Portal or splenic vein thrombosis
• Splenic artery aneurysm
• Hepatic schistosomiasis
• Portal vein obstruction, intrahepatic or extrahepatic
• Cavernous transformation of the portal vein
• Hepatic echinococcosis
• Splenic vein obstruction

14. Reticuloendothelial system hyperplasia
(for removal of defective erythrocytes)
• Spherocytosis
• Early sickle cell anemia
• Ovalocytosis
• Thalassemia major
• Hemoglobinopathies
• Paroxysmal nocturnal hemoglobinuria
• Pernicious anemia
Inflammatory/autoimmune
(Disordered immunoregulation)
• Systemic lupus erythematosus
• Felty syndrome
• Juvenile rheumatoid arthritis
• Hemophagocytic syndrome
• Common variable immunodeficiency
• Immune hemolytic anemias
• Immune thrombocytopenias
• Immune neutropenias
• Drug reactions
• Sarcoidosis
• Thyrotoxicosis

15. Infiltration of the Spleen
(Due to Intracellular or extracellular
depositions)
• Amyloidosis
• Gaucher’s disease
• Niemann-Pick disease
• Tangier disease
• Hurler’s syndrome (mucopolysaccharidoses)
Benign and malignant cellular infiltrations
• Leukemias (acute, chronic, lymphoid, myeloid,
monocytic)
• Lymphomas
• Hodgkin’s disease
• Myeloproliferative syndromes (polycythemia
vera) essential thrombocytosis)
• Angiosarcomas
• Metastatic tumors (melanoma is most common)
• Eosinophilic granuloma
• Histiocytosis X
• Hamartomas
• Hemangiomas, fibromas, lymphangiomas
• Splenic cysts

16. Extramedullary hematopoiesis
• Myelofibrosis
• Thalassemia major
• Marrow damaged by toxins, radiation, strontium
• Marrow infiltration (tumors, leukemias, Gaucher)
Secondary to hemolysis
• Thalassemia major
• Pyruvate kinase deficiency
• Hereditary spherocytosis
• Autoimmune hemolytic anemia

17. MILD SPLENOMEGALY (1-3CM)
just palpable
Acute malaria & Kala-azar
Typhoid
ITP
Felty
hemolytic anemia
viral hepatitis
subacute bacterial endocarditis,
Leptospirosis
Infectious mononucleosis
Acute leukaemias,

18. MODERATE SPLENOMEGALY
(4-8cm) b/w costal margin & umbilicus
Chronic malaria & kala-azar,
Portal Hypertension
CLL
Lymphoma
SLE

19. MASSIVE SPLENOMEGALY - beyond
umblicus, crosses mid line into pelvis
Size >8cm & weighs >1000 g.
• Chronic kala-azar & malaria
• Tropical Splenomegaly syndrome
• Thalassaemia major
• CML & CLL
• Lymphoma including Hairy Cell Luekemia
• Polycythemia vera
• Cirrhosis
• EHPVO
• Myelofibrosis
• Gaucher
• Amyloidosis
• Splenic tumour or abscess
• splenic hemangiomatosis

20. STEP-WISE APPROACH TO SPLENOMEGALY
• History
• Physical examination
• Laboratory testings
• Imaging
• Specialised testing

21. History
• relevant travel history (for example, to tropical areas) (malaria Kala Azar
• family history (for example, Gaucher's disease, hereditary spherocytosis)
• Age
• h/o recent infections like malaria
• Fever,weight loss,sweating (lymphomas, infections)
• Pruritis (PBC,
• Abnormal bleeding/bruising (ITP)
• Joint pain (SLE, RA,
• h/o alcoholism (Cirrhosis, Portal Htn,
• h/o trauma (splenic rupture)
• h/o neonatal umblical sepsis (CVID, Immune Neutropenia, splenic vein thrombosis)
• Jaundice
• High risk sexual behavior (AIDS)
• Past medical history
• Drugs (GM-CSF, RhoGAM, oxaliplatin,

22. PHYSICAL EXAMINATION
 Size of the spleen
 Hepatomegaly
 Lymphadenopathy
 Fever
 Icterus
 Bruising,petechiae
 Oral & supf.sepsis
 Stigmata of liver disease
 Stigmata of RA/SLE
 Splinter hemorrhage,retinal hemorrhage
 Cardiac murmurs

23. LAB INVESTIGATIONS
CBC
Blood smear
Retic count
Blood C/S
Serology (fungal,viral,parasitic)
LFT
Hb electrophoresis
coombs test
Coag.profile
AMA, Anti CCP,RA factor
Bone marrow analysis

24. • High ESR- connective tissue disorder
• Erythrocyte counts may be normal, decreased (thalassemia major syndromes, SLE, cirrhosis with portal hypertension), increased
(polycythemia vera)
• Granulocyte counts may be normal, decreased (Felty’s syndrome, congestive splenomegaly, leukemias), or increased
(infections or inflammatory disease, myeloproliferative disorders
• CBC may reveal cytopenia of one or more blood cell types , which should suggest hypersplenism.
• Leukopenia- feltys syndrome, septicemia
• Blood culture —Often informative in SBE, enteric fever
• Thrombocytopenia— Acute leukaemia, SBE. Blast crisis of CML and CLL, ITP, Felty
• Blood smear—For malaria, kala-azar (buffy coat preparation), leukaemia (acute & chronic), hereditary spherocytosis, thalassaemia
• Paul-Bunnell test - for infectious mononucleosis,
• Serum iron - increased inthalassaemia, haemochromatosis. (iii) Serum copper - low in Wilson’s disease
• Serum bilirubin for cirrhosis with hepato-cellular failure, haemolytic anaemias, malaria, viral hepatitis.
• HPLC- increased HbF in thalassaemia.
• Congo red test for amyloidosis.
• Aldehyde test - for chronic kala-azar.
• Red cell survival time (NESTROFT) - decreased in hereditary spherocytosis,
• Antinuclear factor - positive in SLE.
• Rose-Waaler test - positive in Felty’s syndrome
• Blood for HBsAg. (xiv) Serology—for CMV, EBV, HIV, and VDRL

25. IMAGING
USG- sensitive & specific non-invasive (normal in size if its length is <13 cm or thickness is ≤5 cm)
CT scan/ PET CT
spleen is enlarged if its length is >10 cm
– etiology of splenomegaly
- liver size,heterogenecity
- splenic mets, abscess,calcifcation,cysts
- retro peritoneal LN
- craniocaudal ln > 10 cm
Liver- spleen colloid scan- (RBC –Cr51,Tc99)
considered enlarged if the posterior length is >14 cm, or if the lateral scan area exceeds 80 cc
- hepatic steatosis, SOL ,splenic functions
MRI/ Doppler usg-
portal/splenic vein thrombosis
- cavernomas

26. SPECIALISED TESTING
Abd.fat pad aspiration
JAK-2 mutation (poly Vera)
Gene testing(bcr-abl
Enzyme testing
Lymph node biopsy
FNA/ Biopsy spleen
Diagnostic Splenectomy
Lung or skin biopsy
Liver biopsy

27. SPECIAL SITUATIONS ASSOCIATED WITH SPLENOMEGALY
• Fever- typhoid,malaria,kalaazar, infective endocarditis,leukemia,lymphoma, Collagen vascular diseases ,
Miliary tuberculosis , Acute viral hepatitis.
• Tender spleen- rupture, abscess, infarct, severe congestive right heart failure
• a/c illness+ anemia- AIHA,leukemia
• Anemia- hemolytic anemia,hemoglobinopathies, Severe iron deficiency anaemia, Lymphoma, Leukemia
• Jaundice – cirrhosis,hemolytic anemia, falciparum,
• Pulsatile spleen- aneurysm
• splenomegaly with lymphadenopathy:
Lymphoma, Leukaemia (ALL and CLL), Disseminated tuberculosis, SLE, Sarcoidosis, Felty’s syndrome infectious
mononucleosis

28. butterfly rash in SLE.
Spider naevi and palmar erythema (hepato-cellular failure from cirrhosis of liver).
Kayser-Fleischer ring in cornea (Wilson’s disease).
Tremor (Wilson's disease).
Pigmentation (haemochromatosis c282y mutation).
Leg ulcers (congenital haemolytic anaemia).
Sternal tenderness (acute leukaemias and CML).
Haemorrhagic spots (acute leukaemias, SBE).
Fundoscopy—Roth spots (SBE), choroidal tubercle inmiliary tuberculosis

29. Issue of splenectomy
Indications for splenectomy.
• Splenic rupture
• Some cases of:
• Chronic immune thrombocytopenia
• Haemolytic anaemia, e.g. hereditary spherocytosis,
• autoimmune haemolytic anaemia, thalassaemia major
• Chronic lymphocytic leukaemia and lymphomas
• Primary myelofibrosis
• Tropical splenomegaly
Vaccination against H. influenzae, N. meningitidis, and S. pneumoniae
Complication
• Postoperative thrombocytosis can occur, and aspirin (one lowdose aspirin per day) should be
considered if the platelet countis >1,500 × 109/L
• portal venous thrombosis
• overwhelming post-splenectomy infection/sepsis (OPSI / OPSS)

30. Radionuclide Studies
isotope-labelled, autologous red cells measurement of the splenic red cell pool. The size of the splenic red cell pool should be taken
into account when assessing the significance of anaemia in the presence of splenomegaly. Measuring the pool is particularly useful for
distinguishing polycythaemia vera (increased pool) from secondary (normal pool)
Identification of sites & quantification of red cell destruction. Surface counting over the spleen, heart, and liver following injection of
autologous 51Cr-labelled erythrocytes provides a
qualitative indication of splenic red cell destruction in various haemolytic anaemias
quantitative scanning provides a more accurate measurement of the actual proportion of the cells that are destroyed in the spleen and
elsewhere. These studies are moderatelypredictive of the outcome of splenectomy
Identification and quantification of splenic extramedullary erythropoiesis.
Normally, transferrin-bound iron passes to the bone marrow, where the iron is released and enters erythroblasts for incorporation into
the haemoglobin of developing erythrocytes. In the normal spleen, iron does not dissociate from transferrin.
Hence, the uptake of iron demonstrable by surface counts shortly after administration of radioactive iron ( 59Fe or 52Fe), indicates
Extramedullary erythropoiesis invspleen
Extramedullary erythropoiesis in the spleen occurs in myelofibrosis and essential thrombocythaemia,
but not in patients with polycythaemia vera.
52Fe studies are useful for detecting early stages of transition from polycythaemia vera to myelofibrosis and
for diagnosing the syndrome of transitional myeloproliferative disorder.
Extramedullary haemopoiesis can be accurately identified in thalassaemia major or intermedia and sickle-cell disease by PET after 52Fe
administration, for example paraspinal, mediastinal,
or in lymph node

31. Thank you

32. Acute splenic sequestration crisis (ASSC)
acute drop in Hb level caused by vaso-occlusion within the
spleen and splenic pooling of RBCs. A large percentage of the
total blood volume become sequestered in the spleen, leading
to hypovolemic shock and death.typically occurs in individuals
whose spleens have not yet become fibrotic due to repeated
splenic infarction. Infants with homozygous sickle mutation
(HbSS) or sickle beta0 thalassemia are most often affected, as
well as children or adults with some residual splenic function in
the setting of variant sickle cell syndromes such as HbSC
disease or sickle beta+ thalassemia. Parvovirus B19 infection
may be a risk factor

33. Tropical splenomegaly syndrome 'big spleen disease‘
In areas where malaria is endemic, adults may present with moderate to massive splenomegaly
no obvious signs of active malaria
but all the features of hypersplenism including pancytopenia, expanded plasma volume, and haemolysis.
serum IgM level is usually high, and malarial antibody titres are raised.
spleen shows diffuse proliferation of macrophages.
The relationship to malaria is evident by the response to long-term antimalarial treatment, which produces a
sustained reduction in spleen size and reversal of the cytopenias.

34. HYPERSPLENISM
splenic hyperactivity (splenomegaly) with increased blood cell destruction (cytopenias)
usually accompanied by marrow hyperplasia of the affected cell precursors
There can be a disproportional decrease in the platelets, WBCs & RBCs
thrombocytopenia & leukopenia can be disproportionate to the anemia.
Acute splenic sequestration crisis (ASSC)
• acute drop in Hb level caused by vaso-occlusion within the spleen and splenic pooling of RBCs. A large
percentage of the total blood volume become sequestered in the spleen, leading to hypovolemic shock and
death.typically occurs in individuals whose spleens have not yet become fibrotic due to repeated splenic
infarction. Infants with homozygous sickle mutation (HbSS) or sickle beta0 thalassemia are most often affected,
as well as children or adults with some residual splenic function in the setting of variant sickle cell syndromes
such as HbSC disease or sickle beta+ thalassemia. Parvovirus B19 infection may be a risk factor