Spleen Flashcards
What are the results of splenectomy/hyposplenism?
Transient increase in platelet, RBC, and WBC; persistent increase in lymphocytes and monocytes.
What is the minimum age for splenectomy?
5 years old.
What causes ITP?
IgG antibody to platelet glycoprotein, specifically fibrinogen receptor GpIIb/IIIa and Gp Ia/iia. Results in decrease platelets.
Zdx: petechiae, gingival bleeding, bruising, soft tissue ecchymosis
What is the spleen’s condition in ITP?
The spleen is normal, with no splenomegaly.
How is ITP diagnosed?
Diagnosis of exclusion; requires peripheral smear and bone marrow biopsy, both of which will be normal.
How do children typically present in ITP?
Usually with a preceding viral illness, and most are managed with medical treatment.
In children < 10, usually resolves on its own. Try to avoid splenectomy
What is the treatment for ITP?
Only need to treat if actively bleeding or platelets < 30k
- 1st start with steroids and +/- IVIG/immunoglobulin (Platelets only given if actively bleeding)
- Only 20% will achieve a durable result from above
- 2nd line:
- romiplostim, eltrombopag = thrombopoetin receptor agonist
- Splenectomy
- Rituximab, only tested after splenectomy failure
When is splenectomy indicated for ITP?
Indicating for splenectomy: medical failure (4-6 weeks), prolonged use of steroids (3 months) and platelets < 30k, cases of first relapse, severe life-threatening bleeding
What should be checked if persistent thrombocytopenia occurs after splenectomy?
Look for Howell-Jolly bodies, Heinz bodies, etc. If not present, consider an accessory spleen.
What is TTP and its treatment?
-Thrombotic thrombocytopenic purpura (TTP)
-FAT-RN mnemonic (Fever, Anemia, Thrombocytopenia, Renal, Neurological)
-Caused by defective ADAMTS13 metalloproteinase (vWF cleaving protein) -> Platelet aggregation in microvasculature
-Tx = Plasmapheresis
What is hereditary spherocytosis?
Autosomal dominant
-MC congenital hemolytic anemia requiring splenectomy
-Spectrin membrane protein deficit -> less deformable RBCs & splenic sequestration
Hemolytic anemia
Leg ulcers
Hypersplenism, Pigmented stones, splenomegaly
Tx: splenectomy and cholecystectomy (if has stones) is curative
What should be searched for during splenectomy in hemolytic anemias?
Accessory spleen.
What is pyruvate kinase deficiency?
A congenital hemolytic anemia with altered glucose metabolism; RBC survival is enhanced by splenectomy.
What is warm antibody type acquired immune hemolytic anemia?
IgG against RBCs; splenectomy if refractory to steroids. Will have a positive direct Coombs test.
What is beta thalassemia?
A condition due to persistent HgbF, causing pallor, retarded body growth, and head enlargement; splenectomy if splenomegaly is present.
Most die teens 2/2 hemosiderosis.
Medical tx: blood transfusions, iron chelators (deferoxamine, deferiprone)
What conditions almost always require splenectomy?
Hereditary spherocytosis, elliptocytosis, echinococcal cyst, dermoid cyst, and multiloculated splenic abscess.
What conditions usually require splenectomy?
Refractory warm antibody hemolytic anemia, refractory ITP, isolated splenic lymphoma (with splenomegaly), and myelofibrosis with splenic myeloid metaplasia (spleen acts as bone marrow, Tx: splenectomy if transfusion dependent or severe thrombocytopenia)
What should be done for asymptomatic splenic cysts < 4 cm?
Leave them alone; they can secrete CA 19-9 and CEA but are still benign.
What should be done if an echinococcal cyst is suspected?
Get serologic testing; splenectomy may be needed, and alcohol can be injected before splenectomy.
What is the diagnosis process for Hodgkin’s disease?
Never do FNA; use core needle or excisional biopsy only, along with bone marrow biopsy.
What imaging is used for Hodgkin’s disease?
PET or gallium MRI of spleen and liver; CT does not detect spleen or liver involvement.
What are the stages of Hodgkin’s disease?
Stage I: 1 LN region contiguous;
Stage II: > 2 non-contiguous LN regions on the same side of the diaphragm
Stage III: involved on each side of the diaphragm
Stage IV: Mets to non-lymphoid organs. (liver, bone, lung; not spleen)
Reed-Sternberg cells
A: asymptomatic
B: symptomatic (night sweats, fever, weight loss)
MCC of chylous ascites: lymphoma
What is the prognosis for lymphocyte predominant and lymphocyte depleted Hodgkin’s disease?
Lymphocyte predominant has the best prognosis; lymphocyte depleted has the worst prognosis.
What is the most common type of Hodgkin’s disease?
Nodular sclerosing.