Note-Splenomegaly from any cause can be associated with anemia due to
increased splenic sequestration of RBCs
(as well as other formed blood elements)
I. Most occurrences of splenomegaly result from one of seven
of cells of the RES system or lymphoid lines.
- Acute systemic infections, e.g.
infectious endocarditis, tuberculosis, infectious
mononucleosis, and histoplasmosis
- Autoimmune disease, e.g. Felty's Syndrome and SLE
- Passive congestive splenomegaly
- Usually from hepatic disease, especially cirrhosis (Banti's
Syndrome) with portal hypertension.
of the splenic or portal veins and cor pulmonale can have
the same effect.
- Reactive hyperplasia of the RES cells of
the red pulp can occur,
adding further to the size of the spleen.
- Usually asymptomatic.
- Trapping of erythrocytes in red pulp sinusoids
- Produces congestive splenomegaly
- Causes include:
- Abnormal RBC morphology (especially hereditary
spherocytosis, thalassemias, and early sickle cell anemia)
- Polycythemia vera
- Splenic hematopoiesis
- Occurs in states of bone marrow
insufficiency, most commonly resulting from myeloid
metaplasia and the myelophthisic syndromes
- Anemia is generaly present.
- Primary to the spleen, most commonly:
(Hodgkin's and non-Hodgkin's)-Generalized lymphadenopathy is often
- Chronic lymphocytic and
granulocytic leukemias-Generalized lymphadenopathy is often present
- Acute leukemias.
- Splenic infiltration
- "Storage diseases" (e.g. Gaucher's,
complicated by reactive hyperplasia of red-pulp
- Non-neoplastic space-occupying lesions
granulomas (in mycobacterial and fungal disease)
- Non-infectious granulomas (e.g. in sarcoidosis)
- Infarcts (e.g. in sickle cell anemia, due to ischemia from blockage of splenic sinusoids with deformed
RBCs; also cardioemboli, usually from valvular
vegetations in SBE or from mural thrombi.
99Tc-radionuclide scanning of the spleen is useful in
identifying areas of infarct).
II. Clinical evaluation of a patient with splenomegaly
- In the setting of acute illness, splenomegaly is likely
to be due to reactive hyperplasia or space-occupying
lesions. The spleen tends to be soft on palpation, with
- In chronic illness, the mechanisms of congestion, splenic
hematopoiesis, malignancy, or infiltrative processes are
more probable. A hard, sharp-edged spleen is often felt.
- Presentation of splenomegaly with left-upper quadrant
pain and splenic tenderness suggests infarction,
subcapsular hematoma, or rupture due to trauma or
infection (infectious mononucleosis, typhoid fever, and
malaria are known to cause splenic rupture).
- Asymptomatic splenomegaly is commonly due to passive
- Fever accompanying splenomegaly points to an infectious
etiology; fever, rash, and arthralgias, however, suggest
- 99Tc-radionuclide scan--most useful in identifying
- CT scanning
- Bone marrow aspiration can identify many of the causes of
splenomegaly, including leukemia, lymphoma, lipid storage
diseases, amyloidosis, disseminated fungal or
mycobacterial disease, and metastatic cancer.
- In some cases, diagnostic splenectomy is the only way to
identify the cause of splenomegaly.
IV. Adams' classification of the causes of splenomegaly by the
degree of splenic enlargement is considered to be a useful tool
in narrowing the enormous differential in hard-to-diagnose cases.
This classification is noteworthy in that causes of splenomegaly
which share the same mechanism (e.g. CHF, hepatic cirrhosis, and
HPV obstruction) do not fall into the same category of degree of
- Chronic passive congestion secondary to CHF
- Acute malaria
- Typhoid fever
- SLE, RA
- Thalassemia minor
- Hepatic cirrhosis
- Lymphoma, leukemia
- Infectious mononucleosis
- Hemolytic anemia
- Splenic abcess/infarct
- Gaucher's, Neimann-Pick's
- Hepatic portal vein obstruction
- Thalassemia major
- Chronic malaria