Splenic Pathology

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Pathologies of the Spleen

Spleen in involved in all systemic inflammation, generalized hematopoetic disorders, and many metabolic disturbances... true or false?

True.

Is it common for a disease to originate in the spleen?

No.

When does splenomegaly develop?

Whenever there is a systemic disease.

-LUQ pain
-LUQ fullness
What are these clinical symptoms of?

Splenomegaly.

What are the 2 types of splenic congestion?

Acute- Moderately enlarged spleen
Chronic- Diffusely enlarged spleen

Congestion of the spleen causes dilation of the splenic hilum veins associated with ______________.

Splenomegaly

What are splenic storage diseases?

Amyoloidosis
Gaucher's Disease
Neimann-Pick Disease

What is the protein associated with bone marrow that can be abnormally deposited in excess in any tissue or organ?

Amylid

What is the most frequently involved organ in amyloidosis? What happens with this disease?

Spleen. Amylid builds up in organs.

What are the 2 types of Amyloidosis?

Nodular- Amylid is found in walls of sheathed arteries
Diffuse- Follicles are not involved
Red pulp is involved
Spleen is greatly enlarged and firm

Gaucher's Disease occurs when...

... fat and proteins are deposited abnormally in the body

Where can Gaucher's disease occur?

-liver
-lungs
-spleen
-bone marrow
-less commonly in brain

Interferes in normal function of organss

Who does Gaucher's Disease affect?

Children. 50% of patients are under age 8.

What are clinical findings of Gaucher's disease?

-bone pain
-changes in skin pigmentation

-multiple nodules
-associated with fibrosis
-splenomegaly
-diffuse echogenecity of organ

Gaucher's disease

What is a very fast and fatal storage disease that affects mostly female infants?

Niemann-Pick Disease

What are some clinical findings of Neimann-Pick disease?

-Hepatomegaly
-Digestive disturbances
-Lymphadenopathy

What is a type of lysosome storage disease?

Niemann-Pick disease.
-Lack of enzymes that normally eliminate unwanted substances in the cells in the body

What is a major symptom of Niemann-Pick disease?

-Very enlarged spleen
-Lipid accumulation

What are diffuse diseases?

-Sickle Cell Anemia
-Congenital Spherocytosis
-Hemolytic Anemia
-Polycythemia Vera
-Thalassemia
-Granulocytopoietic Abnormalities
-Reticuloendotheliosis

What is a common disease among African Americans in the US?

Sickle Cell Anemia

What contains an abnormal type of hemoglobin and are misshapen red blood cells?

Sickle cell anemia

Can sickle cell anemia cause infarction?

Yes.

In acute sickle cell crisis...

...sickle cells can interfere with O2 transport, obstruct cappilary flow and causes severe pain. Splenomegaly and decreased hematocrit.

Congenital Shpherocytisis is what?

Intrinsic abnormality of red cells. RBCs are shaped like spheres instead of discs.

What is the normal treatment of Congenital Spherocytisis?

Splenectomy after age 5

Hemolytic Anemia is...

General term applied to a decreased life of erythrocytes.

What is polycythemia vera?

Increased RBC production and hemoglobin concentration.
-Excess production of WBCs, RBCs, and platelets.

What disease is rare and occurs mostly in men over the age of 40?

Polycythemia Vera.

What is a disease that tinnitus is a symptom?

Polycythemia vera.

What is a treatment of polycythemia?

Phlebotomy decreases blood thickness. One pint of blood is removed each week until hematocrit level is less than 45.

What happens with thalassemia?

It is a genetic condition that the spleen is very involved in. The body has difficulty MAKING hemoglobin.

What do cells look like with thalassemia?

Erythrocytes with a central spot of color in the area of pallor, resembling a target.

What are some symptoms of Thalassemia?

-Mild anemia
-Slowed growth
-delayed puberty
-Bone problems
Enlarged spleen, heart and liver

What are some treatments for Thalassemia?

-Blood transfusion
-Folic acid suppliments

What does thalassemia present as sonographically?

-severely enlarged spleen
-appears similar to liver tissue

What disease has problems creating WBCs?

Granulocytopoietic abnormalities

Why would there be problems cerating white blood cells?

From acute or chronic infections

Sonographically what can granulocytopoietic abnormalities look like?

-diffusely hypoechoic pattern
-Pts with previous infections may have echogenic lesions without shadowing
-calcium may be found in splenic artery

What is reticuloendotheliosis?

Varying degrees of lipid storage in phagocytes

How will the spleen appear with reticuloendotheliosis?

Isoechoic.

What is also known as Letterer-Siwe Disease?

non-lipid reticuloendotheliosis

What is letterer-siwe disease?

Proliferation of reticuloendothelial calls in all tissues.
Most commonly seen in pts under 2 years old

Is Hand-Schuller-Christian disease malignant or benign?

Benign.

What is Hand-Schuller-Christian disease?

Lipids accumulate in the body and manifest as granulomas in bone (particularly in skull), skin, viscera.

What gives wheel-within-wheel patterns?

Splenic infections.
-Outer wheel represents the ring of fibrosis surrounding the inner echogenic wheel of inflammatory cells and a central hypoechoic area

What is the most common finding of the spleen with AIDS?

Splenomegaly

What is the most common cause of splenic lesions?

Splenic infarction

What is ALWAYS present with splenic infarction?

Peripheral wedge shaped, hypooechoic lesion base towards subcapsular surface.

What are the varied echogenecities that present with splenic infarction?

Fresh- Localized hyperechoic area
Old, healed- hypoechoic
May be nodular

What is the most commonly injured organ as a result of trauma?

Spleen

What are the 2 outcomes of the spleen with blunt trauma?

-capsule intact
-capsule ruptured

What is it always important to check for with cases of splenic trauma?

Fluid collections.

What is most likely the problem?
-LUQ pain
-Left shoulder pain
-Left flank pain
-Dizziness
-Tenderness over LUQ
-Hypotension
-Decreased hemoglobin
-Splenomegaly most common finding sonographically

Splenic Trauma

What is not a reliable marker of splenic trauma?

Blood because it can have varying appearances.

What are some benign splenic neoplasms?

Hamartoma
Cavernous Hemangioma
Cystic Lymphangioma

What are malignant splenic neoplasms?

-Hermangiosarcoma
-Lymphoma
-Metastases

What resembles cavernous hemangioma?

Hemangiocarcoma

Which has a higher survival rate? Hogkin's or Non- Hodgkin's?

Non-Hogkins Lymphoma

Malignant Splenic Metastases result in the spread of disease from where? (Top 3)

-Breast
-Lung
-Ovary

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