Module 12: Pathophysiology of the Spleen and Adrenal Glands

Define congestive splenomegaly

Enlarged spleen caused by VENOUS outflow obstruction

Name the 2 types of congestive splenomegaly

Acute and chronic

Define acute splenomegaly

Mild to moderate splenomegaly

Define chronic splenomegaly

Diffuse splenomegaly

Name the common causes of congestive splenomegaly

•Portal HTN/cirrhosis

•Heart Failure

•Portosplenic vein thrombosis

•CF

•HIV/AIDS

How would a pt with congestive splenomegaly present?

LUQ pain and palpable mass

Describe the U/S features of congestive splenomegaly.

•Variable echogenicity

•Increased size

•Extends beyond the LK

Name the 2 types of splenic infection/inflammation

Systemic and focal

What are systemic causes of splenic infection/inflammation?

•Mononucleosis

•TB

•Histoplasmosis

•Schistosomiasis

•Sarcoidosis

•Candidiasis

What are focal causes of splenic infection/inflammation?

•Abscess

•Echinococcus

How would a pt with systemic infection/inflammation present?

Variable presentation

Describe the U/S features of systemic infection/inflammation.

•Variable dependent on type

•Normal to increase size

How would a pt with focal splenic infection/inflammation present?

LUQ pain, fever

Describe the U/S features of focal infection/inflammation.

•Focal mass of varying echogenicity

•Irregular borders

•Normal to increase size

•Ill-defined, thickened borders

•Possible acoustic enhancement

•Shadow from presence of gas in the abscess

Define splenic hamartoma

Benign tumor consisting of lymphoid tissue or a combination of sinuses and normal splenic tissue

T/F. Splenic hamartoma is the most common benign tumor found in the spleen.

False. Rare

How would a pt with a hamartoma present?

Asymptomatic

Describe the U/s features of a splenic hamartoma.

•Focal well-defined mass

•Variable echogenicity, typically hyperechoic

•Solid and cystic components

T/F. Hemangioma are the most common benign tumor found in the spleen.

True

Define a splenic hemangioma

Benign tumor comprised of blood vessels found in the spleen.

Where else can a hemangioma be found?

Liver

How would a pt with a splenic hemangiomas present?

•Asymptomatic

•LUQ pain with increased size and/or rupture

Describe the U/S features of a hemangioma.

•Typically, small

•Focal

•Well-defined borders

•Variable echogenicity

•Often hyperechoic

•Hypoechoic areas within

Define leukemia

Cancer of the blood

How would a pt with leukemia present?

•Fever, chills

•Fatigue

•Weight loss

•Bruising

T/F. Treatment of leukemia includes a splenectomy.

True

Define lymphoma

Cancer of the lymphatic system

Which types of lymphoma are assocwith the spleen?

Hodgkin’s and non-Hodgkin’s lymphoma

How would a pt with lymphoma present?

•Weight loss, anorexia

•N/V

•Chronic pain

•Abdominal fullness

Describe the 4 different U/S patterns of lymphoma: 

•Pattern 1

•Pattern 2

•Pattern 3

•Pattern 4

•Diffuse splenic involvement

•Focal, small nodular lesions

•Focal, large nodular lesions

•Bulky disease

Define hemangiosarcoma

Tumor arising form the vascular endothelium of the spleen

T/F. Hemangiosarcoma are common

False, rare

How would a pt with hemangiosarcoma present?

•Weight loss

•Arrythmias

•Lethargy

Describe the U/S features of a hemangiosarcoma

•Mixed cystic pattern

•Focal, well-defined mass

•Hyperechoic

•Variable size

•Resembles the appearance of a cavernous hemangioma

Define splenic METS

Secondary malignancy site

Splenic METS are the result of:

Hematogenous spread from another primary site

Splenic METS are the _____ common site of cancer spread.

10th

Name the common primary cancers that spread to the spleen.

•Breast

•Lung

•Ovary

•Stomach

•Colon

•Kidney

Describe the U/S features of splenic METS

•Multiple focal masses

•Variable size and echogenicity

•Well-defined

•Target lesions, halo lesions

Name the types of splenic cysts

•Congenital

•Post-traumatic

•Infection

•Parasitic

•Neoplastic

How would a pt with a splenic cysts present?

•Asymptomatic

•Pain if rupture or increased size

Describe the U/S features of a splenic cyst.

•Variable, related to types and causes

•Well-defined walls

•Anechoic, hyperechoic

•Internal echoes

•Posterior enhancement

•Fluid-filled level

•Wall thickening, poss. wall calcifications

Define hemolytic anemia

Decreased RBC’s

Define extramedullary hematopoiesis-myelofibrosis

Production of RBC’s and myeloid cells outside the bone marrow

Define sickle cell dz

Inherited disorder in which the RBC’s are sickle shaped and are unable to carry the proper amount of O2

Define polycythemia vera

Excess production of RBC’s

Describe the U/Ss feature of acute  sickle cell disease

Splenomegaly

Describe the U/Ss feature of chronic  sickle cell disease

Atrophy

Describe the U/Ss feature of polycythemia vera

Splenomegaly

Name types of storage diseases

•Gaucher disease

•Diabetes mellitus

•Niemann-Pick disease

•Amyloidosis

•Histiocytosis

•Hemochromatosis

Define Gaucher’s dz

Genetic disorder in which your body is missing the enzyme that is responsible from breaking down lipids

How does Gaucher’ dz affect the liver and spleen?

Results in lipid build up

T/F. Gaucher’s dz can affect organs other than the liver and spleen.

True

What age group does Gaucher’s dzaffect most?

All ages, but 50% < 8 yo, 17% < 1yo

How does a pt with Gaucher’s dzpresent?

•Bone pain

•Skin pigmentation changes

Describe the U/S features of Gaucher’s dz.

•Splenomegaly

•Hepatomegaly

•Diffuse homogeneity

•Multiple splenic nodules

•Well-defined lesions (hypo, hyper, mixed)

•Irregular

Define Niemann-Pick dz

Genetic disease in which the body cannot metabolize lipids and cholesterol

What structures does Niemann-Pick dz affect?

Brain, nerves, liver, spleen, bone marrow

T/F. Niemann-Pick dz is an aggressive and progressive, fatal dz

True

How does a pt with Niemann-Pick dz present?

•Hepatomegaly

•Digestive disturbances

•Lymphadenopathy

Whom does Niemann-Pick dz affect?

Females and infants

Define amyloidosis

Disease in which amyloid builds up in organs

T/F. Amyloidosis is a common storage disease that affects the spleen.

False, rare

Name the 2 manifestations of amyloidosis.

Nodular and diffuse

The _____ is the most affected organ in a pt with amyloidosis.

Spleen

Describe the U/S features of the spleen in a pt with nodular amyloidosis.

Normal appearing spleen

Describe the U/S features of the spleen in a pt with diffuse amyloidosis.

Enlarged spleen

Define splenic infarct

Splenic artery occlusion from an embolus

T/F. Splenic infarct is the most common cause of focal splenic lesions.

True

What is the most common cause of a splenic infarct?

Cardiac embolus

Name the risk factors for developing a splenic infarct.

•Embolus: left sided heart valves, septic

•Thrombosis

•Leukemia

•Sickle cell disease

•Lymphomatous disorders

•Sarcoidosis

How would a pt with a splenic infarct present?

•Asymptomatic

•Sudden onset of LUQ pain

Describe the U/S features of a splenic infarct.

•Wedge shaped lesion: base towards subscapular surface

•Variable echogenicity dependent on age and time of infarction

•Poss. small spleen

Define splenic sequestration crisis in sickle cell dz

Condition in which excessive amounts of blood gets “trapped” in the spleen

Whom does splenic sequestration crisis commonly affect?

Children with homozygous sickle cell disease

How would a pt with splenic sequestration crisis present?

Sudden decrease in hematocrit

Describe the U/S features of splenic sequestration crisis in sickle cell dz.

•Poss subacute hemorrhage

•Hypoechoic area along the periphery

When does a splenic trauma occur?

•Poss subacute hemorrhage

•Hypoechoic area along the periphery

How does a pt with a splenic trauma present?

•LUQ pain

•Poss. LUQ palpable mass

•Decrease in hematocrit

Describe the U/S features of a splenic trauma

•Hematoma: subscapular or intraparenchymal

•Blood in the peritoneal/retroperitoneal cavity

•Variable echogenicity dependent upon age/progression

•Enlarged spleen

•Irregular borders

•Left pleural effusion

Describe the appearance of a Grade 1 splenic trauma

•Splenic laceration

•Subcapsular hematoma

Describe the appearance of a Grade 2 splenic trauma

•Splenic laceration

•Intraparenchymal hematoma

•Subcapsular hematoma

Describe the appearance of a Grade 3 splenic trauma

•Splenic laceration

•Intraparenchymal hematoma

•Subcapsular hematoma

•Poss ruptured subcapsular hematoma

Describe the appearance of a Grade 4 splenic trauma

•Laceration

•Vascular injury

•Intraparenchymal hematoma

Describe the appearance of a Grade 5 splenic trauma

•Vascular injury

•Shattered spleen

Name the conditions assoc with hyperfunction of the adrenal gland

•Cushing’s dz

•Adrenogenital (AG) syndrome

•Conn’s dz (aldosteronism)  

Cushing’s dz is characterized by:

Excessive secretion of cortisol

Cushing’s dz is caused by:

•adrenal hyperplasia

•adrenal or pituitary adenoma

•CA

Cushing’s dz results in:

•Obesity in the trunk (extremities thin)

•moon face

•buffalo hump

•HTN

•renal stones

•irregular menses

•psychiatric disturbances

AG syndrome is characterized by:

Excessive secretion of sex hormones

AG syndrome is caused by:

Adrenal hyperplasia or tumor

AG syndrome results in:

•Varying symptoms

•Ambiguous genitalia

•Masculine features in females, prepubescent males will have signs of masculine development

Conn’s dz is characterized by:

Excessive secretion of aldosterone

Conn’s dz is caused by:

Adrenal cortex adenoma

Conn’s dz results in:

•Muscle weakness

•HTN

•abnormal electrogram

Addison’s dz is AKA:

Adrenocortical insufficiency

Addison’s dz is characterized by:

•Atrophy of the adrenal cortex

•Decreased cortisol and some aldosterone

Addison’s dz results in:

•Increased sodium retention

•Tissue edema

•Increased plasma volume

•Increased potassium excretion

•Hyperpigmentation

•Mild alkalosis

•Fatigue muscle and bone weakness

Define adrenal adenoma.

Benign adrenal tumor of epithelial cells

Is an adrenal adenoma a functioning or non-functioning tumor?

Non-functioning