Splenectomy for Hematologic disorders Flashcards
What is the most common indication for splenectomy?
Trauma
What is the pathology of idiopathic thrombocytopenic purpura (ITP)
An immunologic disorder in which antiplatelet antibodies bind platelets.
What is the treatment for ITP?
Medically with steroids and IVIg
When is splenectomy indicated for ITP?
In adults, if no improvement after 8 weeks of steroids or thrombocytopenia recurs after steroid taper. Splenectomy in children with ITP is rarely indicated (70% cases self limited, resolve spontaneously). Intracranial hemorrhage is also an indication.
Is the spleen enlarged in ITP?
No.
Name secondary causes of ITP
HIV, SLE, Antiphospholipid syndrome, hepatitis C, lymphoproliferative disorders
Name the symptoms of ITP
Thrombocytopenia, petechiae, purpura, ecchymosis and bleeding from mucosal surfaces (gingivae, vagina, GU tract, GI tract)
Risk of intracranial hemorrhage in ITP
1% - 2%
What are the clinical signs of thrombotic thrombocytopenic purpura?
Severe thrombocytopenia, anema, fever, renal failure and neurologic complications
Peripheral blood smear showing schistocytes and nucleated RBCs
TTP
First line treatment for TTP
Daily plasmapheresis and FFP transfusions
Are platelet transfusions recommended in TTP?
No, lead to clinical deterioration in patients w TTP
Is splenectomy ever indicated in autoimmune hemolytic anemia?
Yes. For warm autoimmune hemolytic anemias when remission can not be achieved on steroid therapy.
Splenectomy results in a 60 - 80% improvement in anemia with this condition.
Autoimmune hemolytic anemia (warm)
This syndrome is characterized by rheumatoid arthritis, neutropenia, and splenomegaly.
Felty’s syndrome
How is Felty syndrome treated?
Low dose methotrexate, antirheumatic drugs, or GCSF. Splenectomy is reserved for those with severe neutropenia, recurrent infections or failed therapy.
Caused by EBV and patients often experience Raynaud’s phenomenon.
Autoimmune hemolytic anemia (cold agglutinin syndrome)
Is splenectomy indicated in cold agglutinin syndrome?
No, the RBCs are destroyed in the liver and not the spleen; therefore splenectomy is not indicated. Treatment consists of avoiding cold, use of alkylating agents and plasmapheresis.
What is autoimmune hemolytic anemia?
Formation of IgM autoantibodies to RBC antigens, leading to destruction of RBCs.
True or false: Sarcoidosis is only found in the lung.
False, while 90% of the time, sarcoidosis has primary lung involvement, it can effect every organ in the body.
What percentage of patients with sarcoidosis have splenic involvement?
10-15%
Shown to precipitate TTP
Clopidigrel, ticlopidine, and pregnancy
What is the response rate in patients with TTP who undergo splenectomy?
40%
Where do the petechiae seen in TTP ususally manifest?
Lower extremities
What is the most common red blood cell membrane disorder in North America?
Hereditary spherocytosis
Major protein deficiency in Hereditary spherocytosis
spectrin and ankyrin
Patients commonly present with anemia, jaundice, splenomegaly and cholecystitis (with pigmented stones)
Hereditary spherocytosis
Blood smear shows spherocytes and reticulocytes
Hereditary spherocytosis
Which congenital anemia is most likely to require splenectomy?
Hereditary spherocytosis
For patients with gallstones and spherocytosis, when is cholecystectomy recommended?
At time of splenectomy
Does splenectomy cure patients with hereditary spherocytosis?
Yes
In patients younger than 5 years old, should splenectomy be performed for HS?
No. There is significant risk of OPSI.
What is the etiology of hereditary spherocytosis?
Defects in cell membrane proteins lead to deformed RBCs, inability to traverse the splenic pulp, and premature destruction
Is there a role for partial splenectomy?
Yes, in patients younger than 5 years old with HS, and in patients with hypersplenism in Gaucher’s.
True or False: Splenectomy is generally curative for patients with hereditary anemia secondary to cell membrane disorders.
True. Splenectomy is curative of anemia in patients with spherocytosis, elliptocytosis, pyropikilocytosis and hyrdocytosis.
Blood smear showing target cells
Thalassemia
Indication for splenectomy in thalassemia major
Frequent transfusions (>1 / month), severe pain caused by splenic infarct, or have severe thrombocytopenia (<20K)
In hereditary spherocytosis, are patients born with splenomegaly?
No. However, splenomegaly develops by the time patients are one year old.
Defect in thalassemia
Precipitation of excess globin chains (alpha, beta, gamma, delta), leading to premature destruction of RBCs in spleen
What is the pathology of sickle cell disease?
Single amino acid substitution on the beta hemoglobin chain leading to sickling of cell in hypoxic conditions. This limits RBC transport and predisposes to thrombosis of microvasculature.
At what age do patients homozygous for sickle cell often suffer autosplenectomy from repeated microvascular infarcts?
By age 5.
Indications for splenectomy in sickle cell disease
Splenic abscess or acute splenic sequestration
Mortality rate of acute sequestration crisis?
up to 20%
Percent of patients requiring splenectomy for actue sickle cell crisis?
3%
What is medical management of sickle cell crisis?
Adequate hydration, avoidance of hypothermia and pain control
What is Gaucher’s disease?
Familial disorder of abnormal storage of glycolipids into reticuloendothelial cells. Associated with splenomegaly and lymphadenopathy
When is splenectomy indicated in Gaucher’s?
Signs of hypersplenism (thrombocytopenia, anemia and neutropenia). Splenectomy improves thrombocytopenia.
Complications of splenectomy?
Hemorrhage, atelectasis, pneumonia, pleural effusion, subphrenic abscess, pancreatitis, pancreatic fistula, portal vein thrombosis, OPSI, persistence of hypersplenism (unresected accessory spleen)
What does staging laparotomy entail for Hodgkin’s lymphoma?
liver biopsy, splenectomy, removal of enlarged lymph nodes, and sampling from periaortic, mesenteric, and hepatoduodenal lymph nodes
Indications for splenectomy in Hodgkin’s lymphoma?
Symptomatic splenomegaly, or thrombocytopenia/leukopenia that interferes with medical therapy
What is the most common kind of lymphoma?
Non-Hodgkins
Does splenectomy improve patient symptoms in NonHodgkins?
Yes.
In chronic lymphocytic leukemia (CLL), splenectomy improves thrombocytopenia and anemia in what percent of patients?
60-80%
Does splenectomy benefit CLL patients with small spleens compared to those with splenomegaly?
No, they do respond as well.
True or False: Splenectomy is reserved for palliation of splenomegaly in “hairy cell” leukemia.
True. Most patients with hairy cell leukemia respond well to chemotherapy (pentostatin and cladribine) with longer duration than effect from splenectomy.
What percentage of patients with myelofibrosis develop splenomegaly?
Up to 75%
Why can patients with myelofibrosis have thrombocytopenia or thrombosis?
These patients can have both conditions because the presence of a large spleen (removes plts), but extramedullary hematopoiesis also develops in these patients, resulting in increased platelet numbers.
Why is splenectomy performed in patients with myelofibrosis?
To alleviate symptoms due to mechanical effect of massive spleen.
Myelofibrosis is associated with
Esophagogastric varices from portal hypertension, thrombocytopenia, anemia and pain from splenic infarcts.
Do esophagogastric varices resolve after splenectomy for myelofibrosis?
Yes
Patients are at high risk for what after splenectomy for myelofibrosis?
Thrombocytosis and portal vein thrombosis
Drugs that may reduce risk of postoperative thrombotic complications in myelofibrosis
hydroxyurea, aspirin, anagrelide, and platelet apheresis if plt count > 1 million
Treatment for portal vein thrombosis
Heparin infusion acutely, then 6 months warfarin therapy.
Do patients with essential thrombocythemia and polycythemia vera benefit from early splenectomy?
No, splenectomy offers little benefit for these diseases until myelofibrosis has developed.
Preoperative management in patients undergoing planned splenectomy
Vaccinations 2 weeks preop
What are the vaccinations indicated preop for planned splenectomy?
Vaccinations against encapsulated organisms: haemophilus influenza b, polyvalent Pneumococcus, Meningococcus
What are relative contraindications to splenectomy?
Coagulopathy and portal hypertension
Size limit of spleen for laparoscopic splenectomy
Should be less than 20-25cm in length.
During splenectomy for ITP, when should platelets be infused?
After the splenic artery is doubly ligated.
At reexploration for bleeding post splenectomy, what is the most common source identified?
Bleeding along the diaphragmatic portion of the splenic bed.
Most common location for accessory spleen?
Splenic hilum
Incidence of accessory spleens?
30%
Incidence of OPSS (overwhelming postsplenectomy sepsis) in general population?
1-4%
Incidence of OPSS in patients less than 5 with a history of hematologic disorders?
Approaches 12-15% in some reports
Mortality associated with OPSS
50%
