The Spleen

Christine Johns, MD May 2009

Outline
 Normal spleen anatomy  Normal spleen function  Physical exam

 Spleen problems other than splenomegaly
 Splenomegaly

Spleen anatomy—gross
 Next to diaphragm, near 9th, 10th, 11th ribs  Tail of pancreas next to hilum

Spleen anatomy—gross
 Inside the periotenum with peritoneal adhesions

to capsule  Soft, breakable material—can rupture easily

Spleen anatomy—gross
 Red-blue color

 Shaped like coffee bean
 10-13 cm in length  Average weight 150 g

 Surrounded by fibrous capsule
 Splenic artery and vein enter at hilum

Spleen anatomy—gross
 Cut surface deep red color (“red pulp”) with

circular white areas (“white pulp”)

 White pulp is area of lymphoid tissue, can enlarge

when fighting infection (“white pulp hyperplasia”)

Red pulp

White pulp with germinal center

Capsule

White pulp— lymphoid follicle

Germinal center (B cells)

Red pulp

Outline
 Normal spleen anatomy  Normal spleen function  Physical exam

 Spleen problems other than splenomegaly
 Splenomegaly

 White pulp—area of immune reaction (immune

system functions)
 Red pulp—blood filter and storage site

(hematopoietic functions)

Spleen Functions
 Immune functions (25% of lymphoid tissue in

body)
 Makes lymphocytes  Makes antibodies  Activation of lymphocytes  Important to protect against bacteria—especially

encapsulated organisms (pneumococcus, meningococcus, etc.)

Spleen Functions
 Hematopoietic (blood system)
 Remove („eat‟ phagocytose) old blood cells  Remove damaged blood cells (Heinz bodies,    

Howell-Jolly bodies, target cells, spherocytes) Store platelets (normally 30% of platelets) Remove iron from hemoglobin of old RBCs Make blood cells (before birth only) Macrophages compete with bacteria for iron (bacteria cannot grow well without iron)

Howell-Jolly Body
 Piece of nucleus left inside

RBC  Usually removed by spleen  Can use to monitor spleen function
 New Howell-Jolly bodies

Hyposplenism (ex sickle cell)
 Disappearance of Howell-Jolly

bodies  new growth of spleen tissue in (ex. Splenosis)

Spleen examination
 Usually not palpable in adults—sometimes

palpable in cachetic patients and children and teenagers  May feel spleen if too big or firm (not soft)  Usually must be at >40% bigger than normal to palpate, but some very big spleens cannot be palpated

Outline
 Normal spleen anatomy  Normal spleen function  Physical exam

 Spleen problems other than splenomegaly
 Splenomegaly

Spleen examination—palpation
 Make sure patient‟s abdomen is relaxed, arms at side  Relax hand/arm and do not press too hard or too    

suddenly Use left hand to support left lower rib cage, use right hand to palpate Start palpation in right lower quadrant and move toward LUQ May only feel spleen at end inspiration Can turn patient onto right side to bring spleen forward

Spleen exam—percussion
 Castell‟s sign
 Percuss over the last intercostal


 

space on the midaxillary line during full inspiration and full expiration Listen for change from tympanic sound to dull sound with full inspiration  splenomegaly Sensitivity: 82% Specificity: 83% May have false positive if right after eating; difficult if obese

Outline
 Normal spleen anatomy  Normal spleen function  Physical exam

 Spleen problems other than splenomegaly
 Splenomegaly

Hyposplenism or asplenia
 Causes
 Congenital  Sickle cell disease  Post-splenectomy  Post splenic artery thromboisis  Celiac disease or inflammatory bowel disease

 Possible signs:
 Sepsis with encapsulated organisms  Thrombocytosis, leukocytosis  Howell-Jolly bodies, Heinz bodies, target cells

Rupture
 Usually secondary to trauma but can be

spontaneous, especially if splenomegaly  Surgical emergency due to blood loss—spleen is very vascular

Splenic Abscess
 Usually secondary to endocarditis or seeding

from another infection  Findings: LUQ pain, fever, +/- splenomegaly, possible, left-sided pleural effusion, splenic infarct  Treatment: combined antibiotics and splenectomy

Splenic infarct
No blood flow to part of spleen cells die Possible causes:  Hypercoagulable state  Embolic disease  Underlying myeloproliferative disorder with associated (often massive) splenomegaly (eg, polycythemia vera, essential thrombocythemia, primary myelofibrosis)  Underlying hemoglobinopathy, especially sickle cell disease  Any condition associated with marked splenomegaly  Trauma to the spleen which compromises its vascularity

Outline
 Normal spleen anatomy  Normal spleen function  Physical exam

 Spleen problems other than splenomegaly
 Splenomegaly

Splenomegaly symptoms
 Acute: LUQ pain and tenderness (capsule

strectch)
 Chronic, subacute: LUQ fullness, pain radiates to

left shoulder, early satiety (feels full with little food)

Causes
Hyperfunction

Splenomegaly

Abnormal blood flow

Infiltration

Removal of abnormal RBCS

Organ failure

Malignant

Infection

Vascular flow

Non-malignant

Auto-immune

Hematopoiesis

Hyperfunction
 Removal of abnormal RBCs
 Hemoglobinopathies  Thalassemia  Sickle cell disease (early)  Others  G6PD deficiency during oxidation crisis  Hereditary spherocytosis or elliptocytosis

 Other hemolytic anemia of any cause

Normal red blood cells

Hereditary elliptocytosis

Hereditary spherocytosis

Heinz Bodies
 Hemoglobin precipitates  Use special stain to see easily  Seen in G6PD deficiency, α-thalassemia, liver

disease, other hemolytic anemias

“Bite cells”
 Spleen removes Heinz bodies (preciptated

hemoglobin) but damages RBC membrane

Sickle cell disease (not in Lao people)

Target cells
 Also called codocytes  Thin, high surface area to volume ratio  Liver disease, hemoglobin C, thalassemia, iron

deficiency, post-splenectomy or hyposplenism

Pappenheimer bodies
 Iron particles inside RBC  Smaller than Howell-Jolly bodies

Hyperfunction
Infection Reactive white pulp hyperplasia to fight infection
 Bacteria
 Endocarditis,

 Viral

septicemia, splenic abscess  Typhoid fever  Brucellosis  Leptospirosis  Tuberculosis  Mycobacterium avium complex (AIDS)  Syphillis  Chlamydia psittacosis

Mononucleosis (EBV) Acute viral hepatitis Cytomegalovirus (CMV) AIDS Fungal Histoplasmosis Parasites Malaria Leischmaniasis (kala azar) Toxoplasmosis Trypansosomiasis Ehrlichiosis

Infectious mononucleosis--EBV
 Usually young people with fatigue,

lymphadenopathy, fever, sore throat, can have hepatitis, recover in 4-6 weeks  Atypical lymphocytes on smear

Hyperreactive malarial splenomegaly syndrome
 “Tropical splenomegaly”
 Exposure to malaria causes

massive IgM production  Splenomegaly is moderate to massive  Anemia, fatiuge, weight loss, +/abdominal pain  Treat with long term antimalarials

Hyperreactive malarial splenomegaly syndrome
 Major criteria  Gross splenomegaly >10 cm below  Minor criteria  Hepatic sinusoidal lymphocytosis  Normal cellular and humoral

the costal margin in adults, no other cause can be found  Elevated serum IgM >2 standard deviations above the local avgerage.  Clinical and immunologic responses to antimalarial therapy  Regression of splenomegaly by 40% by 6 months after start of therapy  High antibody levels of Plasmodium species (³ 1:800)

responses to antigenic challenge, including a normal phytohemagglutination response  Hypersplenism  Lymphocytic proliferation  Familial occurrence

Hyperfunction—autoimmune
 Rheumatoid arthritis (Felty‟s syndrome)  Systemic lupus erythematosus (SLE)  Sarcoidosis

 Idiopathic immune thrombocytopenia (ITP)
 Autoimmune hemolytic anemia  Serum sickness (drug reaction)

Hyperfunction—hematopoiesis
 Spleen makes blood cells until 5th month of

gestation (before birth), then normally stops.  Later, if bone marrow cannot make blood cells— spleen may make some blood cells
 Examples:  Myelofibrosis (bone marrow scarring)  Bone marrow invaded by tumor or leukemia  Bone marrow suppression because of radiation or toxin

Causes
Hyperfunction

Splenomegaly

Abnormal blood flow

Infiltration

Removal of abnormal RBCS

Organ failure

Malignant

Infection

Vascular flow

Non-malignant

Auto-immune

Hematopoiesis

Abnormal blood flow
 Organ failure
 Cirrhosis with portal hypertension  Congestive heart failure

 Vascular problem
 Spleinic vein obstruction  Portal vein obstruction  Hepatic vein obstruction (Budd-Chiari syndrome)  Portal hypertension due to other causes (hepatic

schistosomiasis, hepatic echinococcus)

Causes
Hyperfunction

Splenomegaly

Abnormal blood flow

Infiltration

Removal of abnormal RBCS

Organ failure

Malignant

Infection

Vascular flow

Non-malignant

Auto-immune

Hematopoiesis

Infiltration
 Malignant
 Leukemia (acute or chronic, lymphoid or myeloid)  Lymphoma (Hodgkin‟s or non-Hodgkin‟s)  Myeloproliferative disorders  Polycythemia vera (too many RBCs)  Essential thrombocytosis (too many platelets)  Myelofibrosis (bone marrow fills with fibrosis-scar and cannot make blood cells)  Metastatic tumors (esp. multiple myeloma)

Infiltration
 Non-malignant
 Metabolic diseases  Storage diseases (Gaucher‟s disease, Niemann-Pick syndrome, Hurler syndrome and other mucopolysaccharide diseases, etc.)  Amyloidosis  Structural and others  Splenic cysts  Hamartomas  Hemangioma  Lymphangioma  Langerhans cell histiocytosis  Eosinophilic granuloma

Most common causes of splenomegaly (in non-tropical countries)
 Liver disease— 33% (cirrhosis)  Hematologic malignancy — 27% (lymphoma)  Infection — 23% (AIDS, endocarditis)  Congestion or inflammation — 8% (congestive

failure)  Primary splenic disease — 4% (splenic vein thrombosis)  Other or unknown — 5%

Causes of massive splenomegaly >1000 g
 Chronic leukemia (especially lymphocytic)  Myelofibrosis (including post-polycythemia vera)  Thalassemia

 Lymphomas (slow growing, including hairy cell leukemia)
 Hyperreactive malarial splenomegaly syndrome (tropical    

splenomegaly) Visceral leischmaniasis (kala azar) Schistosomiasis AIDS with mycobacterium avium complex Gaucher‟s disease and Niemann Pick disease (usually in Jews)

Approach to splenomegaly
 HISTORY! HISTORY! HISTORY!  Physical examination  CBC with WBC differential and peripheral blood

smear
 Normal appearing cells but low numbers?  Immature or abnormal blood cells?

 Micrangiopathic hemolytic anemia?
 Bacteria, parasites?  LE cells?

LOTS of information can be learned from peripheral blood smear. Try to look at it yourself!!!

Approach to splenomegaly
 If still unclear etiology, imaging of abdomen—

ultrasound first, consider CT to look for malignancy if no other cause found  Biopsy of appropriate tissue: lymph node if suspect infection, liver if likely liver disease, bone marrow for hematologic malignancy. If no specific findings, consider bone marrow biopsy and aspirate.  Splenectomy: last choice to make diagnosis, may be therapeutic for some diseases
 Give immunizations for encapsulated organisms

before surgery (Haemophilus influenza, S. pneumo, Neisseria)