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Pathology of the Spleen

Terms in this set (105)

What is the most common disease process of the spleen?
Splenomegaly.
What are the clinical symptoms of splenomegaly?
LUQ pain
LUQ fullness
Enlargement may encroach upon - left kidney, pancreas, stomach and intestines.
What can cause splenomegaly?
Congestion
Neoplasm
Infection
Hemolytic anemia
Extramedullary hematopoiesis
Trauma
What are the two types of splenic congestion?
Acute - moderate enlargement
Chronic - diffusely enlarged
What can congestion of the spleen be caused by?
Cirrhosis of the liver
Intrahepatic obstruction
Portal venous drainage problems
Obstructive venous disorders in the portal and/or splenic veins
Cardia decompensation
Tricuspid or pulmonary valvular disease.
What are the storage diseases of the spleen?
Amyloidosis
Gaucher's Disease
Niemann-Pick Disease
What is the protein that is produced by cells in bone marrow that can be deposited in any tissue or organ, the build-up of which, though rare can be potentially fatal?
Amyloid protein. Amyloidosis.
What are the two types of amyloidosis?
Nodular and diffuse.
What is the sonographic appearance of amyloidosis?
The spleen can be normal to very enlarged in size.
In nodular amyloidosis, what is affected?
The walls of the sheathed arteries and within the follicles.
In diffuse amyloidosis, what is involved?
The red pulp.
What organs can Gaucher's Disease affect?
Liver, spleen, lungs, bone marrow and brain.
What occurs with Gaucher's disease?
Harmful fatty substances accumulate and interfere with organ function causing organ enlargement and bone pain.
What causes Gaucher's disease?
Enzyme deficiency -glucocerebrosidase
What are clinical findings of Gaucher's disease?
Bone pain and changes in skin pigmentation.
What are the sonographic findings of Gaucher's disease?
Splenomegaly
Diffuse inhomogeneity
Multiple nodules - irregular, hyperechoic, mixed.
Niemann-Pick Disease
A lysosome storage disease that affect metabolism and is caused by genetic mutation.
What is abnormal with types A and B Niemann-Pick disease?
There is a deficiency of the enzyme acid sphingomyelinase
What is abnormal with type C Niemann-Pick disease?
Patients are not able to metabolize cholesterol and other lipids
What population does Niemann-Pick disease mostly affect?
Female infants.
What are the clinical findings with Niemann-Pick disease?
Hepatomegaly
Digestive disturbances
Lymphadenopathy
Jaundice
What are the sonographic findings with Niemann-Pick disease?
Extreme splenomegaly
Echogenic lipid accumulation
Various other symptoms
What are the three categories of diffuse diseases that can affect the spleen?
Myeloproliferative disorders
Granulocytopoietic abnormalities
Erythropoietic disease
Myeloproliferative disorders
Acute and chronic myelogenous leukemias
Polycythemia Vera
Myelofibrosis
Megakaryocytic leukemia
Erythroleukemia
Erythropoietic diseases
Sickle cell
Hereditary spherocytosis
Hemolytic anemia
Chronic anemia
Polycythemia vera
Thalassemia
What is abnormal in sickle cell anemia?
The hemoglobin molecule, resulting in crescent shaped RBCs which have a difficult passing through blood vessels.
What are the clinical findings with sickle cell anemia in infants and children?
The spleen is enlarged due to red pulp congestion.
What eventually happens to the spleen with sickle cell anemia.
The spleen undergoes progressive infarction and fibrosis becoming small; eventually autosplenectomy.
What occurs with acute sickle cell crisis?
Oxygen transport and capillary flow, causing severe pain in the joints and the abdomen.
What are clinical findings for acute sickle cell crisis?
Splenomegaly
Sudden decrease in hemocrit
May develop a subacute hemorrhage appearing as a hypoechoic area in the periphery of the spleen.
What is the treatment for sickle cell disease?
Blood and bone marrow transplant.
What is the prognosis for sickle cell disease?
Life expectancy 40s and beyond
Still a sever, chronic and sometimes fatal disease.
This is a disorder of the membrane of the RBC where it is shaped like a sphere.
Congenital spherocytosis.
What effect does congenital spherocytosis have on RBCs.
It leads to their premature breakdown (hemolytic anemia)
What are the clinical findings of congenital spherocytosis in infants?
Jaundice, fatigue, weakness, shortness of breath and irritability.
What are the sonographic findings in congenital spherocytosis?
Enlarged spleen
What is the treatment for congenital spherocytosis?
Splenectomy after the age of 5, only to treat the premature destruction of RBCs - RBCs have a normal life span.
Autoimmune hemolytic anemia
Malfunction of the immune system leading to the production of antibodies which attack RBCs as if they were foreign invaders.
What are clinical symptoms of autoimmune hemolytic anemia?
Can be no symptoms, fatigue, SOB, pale; severe may have jaundice, abdominal discomfort and fullness.
How is autoimmune hemolytic anemia treated?
Corticosteroids (moderate) or immunosuppressive drugs (very severe - post splenectomy); in severe cases splenectomy and
What are the sonographic findings of autoimmune hemolytic anemia?
Splenomegaly
What occurs with polycythemia vera?
Increased RBC production and hemoglobin concentration due to bone marrow producing excessive RBCs, WBCs and platelets.
What population does polycythemia vera typically affect?
Rare, affects men more and is rare under 40.
What is the main cause of symptoms in polycythemia vera?
Increased blood thickness and clotting.
What are the sonographic findings with polycythemia vera?
Variable enlargement of the spleen, firm and infarcts and thromboses are common.
How is polycythemia vera treated?
Remove blood - 1 pt per week until normal.
Thalassemia
A genetic disease involving the spleen; the body has difficulty making hemoglobin. RBCs are deficient in hemoglobin , abnormal in size and shape and have reduced life spans.
How do the erythrocytes appear with thalassemia?
Target cells (codocytes), with a central spot of color in the area of pallor resembling a target.
What are the typical clinical findings with thalassemia?
Mild - no symptoms or minor fatigue.
Moderate - delayed growth and puberty, brittle bones, LUQ fullness
Severe - Pale, poor appetite, dark urine, delayed growth and puberty, jaundice, enlarged spleen, heart and liver, bone problems particularly in the face.
What is the ultrasound finding in thalassemia?
Extreme enlargement of the spleen in severe cases, filling the entire abdominal cavity.
How is thalassemia treated?
Blood transfusion, folic acid supplements
What is the prognosis for thalassemia?
Severe cases can cause early death due to heart failure between 20s and 30s. Less severe forms have little effect on life span.
What does granulocytopoietic abnormality affect?
Creation of WBCs
What is a type of granulocytopoietic abnormality?
Reactive hyperplasia - a result of chronic infection (e.g. splenitis sacroid and tuberosclerosis)
What are the ultrasound findings of granulocytopoietic abnormality?
Splenomegaly
Diffusely hypoechoic pattern
History of infection may have echogenic lesions without shadowing
Calcium may be found in the splenic artery
Reticuloendotheliosis
Proliferation of RE in any organ or tissue; storage of lipids in phagocytes.
Letterer-Siwe Disease
AKA nonlipid reticuloendotheliosis and histocytosis; RE cells proliferates in all tissues, particularly splenic lymph nodes and bone marrow.
What age group does Letterer-Siwe disease mostly affect?
Children under 2.
What are the clinical findings of Letterer-Siwe disease?
Hepatosplenomegaly
Fever
pulmonary involvement
How does the spleen appear sonographically with Letterer-Siwe disease?
Moderately enlarged
What is the prognosis for Letterer-Siwe disease?
Rapidly fatal if untreated
Hand-Schuller-Christian disease
A rare disease resulting in lipid accumulation in the body manifesting as histolytic granuloma in the bones (skull), skin and viscera.
What are the clinical symptoms of Hand-Schuller-Christian disease?
Hepatosplenomegaly
Lymphadenopathy
Diabetes
What population does Hand-Schuller-Christian disease typically affect?
Children over 2 years
What are the sonographic findings associated with Hand-Schuller-Christian disease?
Splenomegaly
How can leukemia affect the spleen?
Moderate to severe enlargement
Why are splenic abscesses rare?
Phagocytic activity and the spleen's efficient RE system and the presence of leukocytes.
What are typical causes of splenic abscesses?
Subacute bacterial endocarditis
Septicemia
Decrease immunological states
Drug abuse
What are the typical US findings with splenic abscess?
Mixed echo pattern
Hypoechoic with a hyperechoic focus
Thick or irregular walls
Anechoic area within a mass
Poor definition of the lesion
Increased through transmission (gas)
How do the spleens of immunocompromised patients with a splenic infection appear?
Multiple nodules
What does hepatosplenic candidiasis look like sonographically?
"wheel within a wheel", bullseye
What is the most common cause of focal splenic lesions?
Splenic infarction caused by an occlusion of the SA or a branch
How does a splenic infarction appear in US?
A peripheral, wedge shaped, hypoechoic lesion
What are the two outcomes of splenic trauma?
Capsule intact or ruptured
Splenic trauma - capsule intact
Intraparenchymal hematoma
Subcapsular hematoma
Splenic trauma - capsule rupture
Focal or free intraperitoneal hematoma.
In the case of splenic trauma, where should you check for fluid?
Around the spleen and then all around the abdomen.
What are sonographic findings associated with splenic trauma?
Splenomegaly
Irregular borders
Hematoma
Contusion
Subcapsular and pericapsular fluid collections
Free intraperitoneal fluid
Left pleural effusion
What are the classifications of splenic cysts?
Parasitic and nonparasitic (primary, secondary and true)
What are most splenic cysts secondary to?
Trauma, infection or infarction.
What is the parasitic infection of the spleen called?
Echinococcus aka Hyatid disease.
How does a Echinococcal cyst appear in US?
Anechoic with possible daughter cysts and calcifications; a solid mass with fine internal echoes and poor posterior enhancement
What are primary splenic cysts?
Epidermoid - contain epithelial lining and congenital in origin. They occur more in femals 15yrs and younger
What are secondary splenic cysts?
aka false cysts; a result of previous trauma, no lining and account for 80% of nonparasitic cysts.
True splenic cysts
Usually solitary and rarely contain calcifications.
What can splenic cysts contain?
Fluid containing:
-protein
-iron
-bilirubin
-fat
-cholesterol crystals
What are Benign splenic neoplasms?
Hamartoma
Cavernous hemangioma
Cystic lymphangioma
What are malignant splenic neoplasms?
Hemangiosarcoma
Lymphoma
Metastases
What is the most common benign neoplasm of the spleen?
Hamartoma
What are hamartomas comprised of?
Vascular and lymphoid tissue
How do hamartomas appear sonographically?
Solid and cystic component, typically hyperechoic.
Cavernous hemangioma
Jumbled growth of blood vessels in spleen and liver
How does a cavernous hemangioma appear sonographically?
Large inhomongenous mass with a small hypoechoic area; complex. An infarction with coagulated blood or fibrin
Cystic lymphangioma
Rare in the spleen; a malformation of the lymphatics; can affect the liver,spleen, kidney and colon.
What does cystic lymphangioma look like sonographically?
Extensive replacement of the splenic parenchyma with cysts
Hemangiosarcoma
Rare, primary cancer composed of the vascular endothelium of the spleen; likely to cause bleeding
What is the sonographic appearance of hemangiosarcoma?
Mixed cystic pattern - resembles cavernous hemangioma
Lymphoma with splenic involvement.
Affects lymphocytes; most common blood CA in the US.
Hodgkin's Lymphoma
Painless swelling of lymph nodes, persistant fatigue, fever, chills, night sweats, unexplained weight loss, loss of appetite, itching
Hodgkin's lymphoma - sonographic findings
Typically hypoechoic
4 patterns:
-diffuse
-small focal lesions
-large focal nodular lesions
-bulky disease
Hodgkin's lymphoma - prognosis
75-80% cure overall; almost 100% in young patients
Non-Hodgkin's lymphoma
5 times more common than Hodgkin's; abnormal lymphocytes are produced that divide and grow uncontrollably, crowd into the lymph nodes causing swelling.
Non-Hodgkin's Lymphoma - symptoms
Swollen, painless lymph nodes
Non-Hodgkin's lymphoma - prognosis
Dependent on stage - 1yr survival 70%, 5yr - 51%
Splenic neoplasms - metastases
From:
-breast
-lung
-ovaries
-stomach
-colon
-kidney
-prostate
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