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Cameron's Esophagus and Spleen > Splenectomy for Hematologic disorders > Flashcards

Splenectomy for Hematologic disorders Flashcards

1
Q

What is the most common indication for splenectomy?

A

Trauma

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2
Q

What is the pathology of idiopathic thrombocytopenic purpura (ITP)

A

An immunologic disorder in which antiplatelet antibodies bind platelets.

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3
Q

What is the treatment for ITP?

A

Medically with steroids and IVIg

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4
Q

When is splenectomy indicated for ITP?

A

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.

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5
Q

Is the spleen enlarged in ITP?

A

No.

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6
Q

Name secondary causes of ITP

A

HIV, SLE, Antiphospholipid syndrome, hepatitis C, lymphoproliferative disorders

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7
Q

Name the symptoms of ITP

A

Thrombocytopenia, petechiae, purpura, ecchymosis and bleeding from mucosal surfaces (gingivae, vagina, GU tract, GI tract)

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8
Q

Risk of intracranial hemorrhage in ITP

A

1% - 2%

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9
Q

What are the clinical signs of thrombotic thrombocytopenic purpura?

A

Severe thrombocytopenia, anema, fever, renal failure and neurologic complications

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10
Q

Peripheral blood smear showing schistocytes and nucleated RBCs

A

TTP

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11
Q

First line treatment for TTP

A

Daily plasmapheresis and FFP transfusions

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12
Q

Are platelet transfusions recommended in TTP?

A

No, lead to clinical deterioration in patients w TTP

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13
Q

Is splenectomy ever indicated in autoimmune hemolytic anemia?

A

Yes. For warm autoimmune hemolytic anemias when remission can not be achieved on steroid therapy.

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14
Q

Splenectomy results in a 60 - 80% improvement in anemia with this condition.

A

Autoimmune hemolytic anemia (warm)

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15
Q

This syndrome is characterized by rheumatoid arthritis, neutropenia, and splenomegaly.

A

Felty’s syndrome

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16
Q

How is Felty syndrome treated?

A

Low dose methotrexate, antirheumatic drugs, or GCSF. Splenectomy is reserved for those with severe neutropenia, recurrent infections or failed therapy.

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17
Q

Caused by EBV and patients often experience Raynaud’s phenomenon.

A

Autoimmune hemolytic anemia (cold agglutinin syndrome)

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18
Q

Is splenectomy indicated in cold agglutinin syndrome?

A

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.

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19
Q

What is autoimmune hemolytic anemia?

A

Formation of IgM autoantibodies to RBC antigens, leading to destruction of RBCs.

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20
Q

True or false: Sarcoidosis is only found in the lung.

A

False, while 90% of the time, sarcoidosis has primary lung involvement, it can effect every organ in the body.

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21
Q

What percentage of patients with sarcoidosis have splenic involvement?

A

10-15%

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22
Q

Shown to precipitate TTP

A

Clopidigrel, ticlopidine, and pregnancy

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23
Q

What is the response rate in patients with TTP who undergo splenectomy?

A

40%

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24
Q

Where do the petechiae seen in TTP ususally manifest?

A

Lower extremities

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25
Q

What is the most common red blood cell membrane disorder in North America?

A

Hereditary spherocytosis

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26
Q

Major protein deficiency in Hereditary spherocytosis

A

spectrin and ankyrin

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27
Q

Patients commonly present with anemia, jaundice, splenomegaly and cholecystitis (with pigmented stones)

A

Hereditary spherocytosis

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28
Q

Blood smear shows spherocytes and reticulocytes

A

Hereditary spherocytosis

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29
Q

Which congenital anemia is most likely to require splenectomy?

A

Hereditary spherocytosis

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30
Q

For patients with gallstones and spherocytosis, when is cholecystectomy recommended?

A

At time of splenectomy

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31
Q

Does splenectomy cure patients with hereditary spherocytosis?

A

Yes

32
Q

In patients younger than 5 years old, should splenectomy be performed for HS?

A

No. There is significant risk of OPSI.

33
Q

What is the etiology of hereditary spherocytosis?

A

Defects in cell membrane proteins lead to deformed RBCs, inability to traverse the splenic pulp, and premature destruction

34
Q

Is there a role for partial splenectomy?

A

Yes, in patients younger than 5 years old with HS, and in patients with hypersplenism in Gaucher’s.

35
Q

True or False: Splenectomy is generally curative for patients with hereditary anemia secondary to cell membrane disorders.

A

True. Splenectomy is curative of anemia in patients with spherocytosis, elliptocytosis, pyropikilocytosis and hyrdocytosis.

36
Q

Blood smear showing target cells

A

Thalassemia

37
Q

Indication for splenectomy in thalassemia major

A

Frequent transfusions (>1 / month), severe pain caused by splenic infarct, or have severe thrombocytopenia (<20K)

38
Q

In hereditary spherocytosis, are patients born with splenomegaly?

A

No. However, splenomegaly develops by the time patients are one year old.

39
Q

Defect in thalassemia

A

Precipitation of excess globin chains (alpha, beta, gamma, delta), leading to premature destruction of RBCs in spleen

40
Q

What is the pathology of sickle cell disease?

A

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.

41
Q

At what age do patients homozygous for sickle cell often suffer autosplenectomy from repeated microvascular infarcts?

A

By age 5.

42
Q

Indications for splenectomy in sickle cell disease

A

Splenic abscess or acute splenic sequestration

43
Q

Mortality rate of acute sequestration crisis?

A

up to 20%

44
Q

Percent of patients requiring splenectomy for actue sickle cell crisis?

A

3%

45
Q

What is medical management of sickle cell crisis?

A

Adequate hydration, avoidance of hypothermia and pain control

46
Q

What is Gaucher’s disease?

A

Familial disorder of abnormal storage of glycolipids into reticuloendothelial cells. Associated with splenomegaly and lymphadenopathy

47
Q

When is splenectomy indicated in Gaucher’s?

A

Signs of hypersplenism (thrombocytopenia, anemia and neutropenia). Splenectomy improves thrombocytopenia.

48
Q

Complications of splenectomy?

A

Hemorrhage, atelectasis, pneumonia, pleural effusion, subphrenic abscess, pancreatitis, pancreatic fistula, portal vein thrombosis, OPSI, persistence of hypersplenism (unresected accessory spleen)

49
Q

What does staging laparotomy entail for Hodgkin’s lymphoma?

A

liver biopsy, splenectomy, removal of enlarged lymph nodes, and sampling from periaortic, mesenteric, and hepatoduodenal lymph nodes

50
Q

Indications for splenectomy in Hodgkin’s lymphoma?

A

Symptomatic splenomegaly, or thrombocytopenia/leukopenia that interferes with medical therapy

51
Q

What is the most common kind of lymphoma?

A

Non-Hodgkins

52
Q

Does splenectomy improve patient symptoms in NonHodgkins?

A

Yes.

53
Q

In chronic lymphocytic leukemia (CLL), splenectomy improves thrombocytopenia and anemia in what percent of patients?

A

60-80%

54
Q

Does splenectomy benefit CLL patients with small spleens compared to those with splenomegaly?

A

No, they do respond as well.

55
Q

True or False: Splenectomy is reserved for palliation of splenomegaly in “hairy cell” leukemia.

A

True. Most patients with hairy cell leukemia respond well to chemotherapy (pentostatin and cladribine) with longer duration than effect from splenectomy.

56
Q

What percentage of patients with myelofibrosis develop splenomegaly?

A

Up to 75%

57
Q

Why can patients with myelofibrosis have thrombocytopenia or thrombosis?

A

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.

58
Q

Why is splenectomy performed in patients with myelofibrosis?

A

To alleviate symptoms due to mechanical effect of massive spleen.

59
Q

Myelofibrosis is associated with

A

Esophagogastric varices from portal hypertension, thrombocytopenia, anemia and pain from splenic infarcts.

60
Q

Do esophagogastric varices resolve after splenectomy for myelofibrosis?

A

Yes

61
Q

Patients are at high risk for what after splenectomy for myelofibrosis?

A

Thrombocytosis and portal vein thrombosis

62
Q

Drugs that may reduce risk of postoperative thrombotic complications in myelofibrosis

A

hydroxyurea, aspirin, anagrelide, and platelet apheresis if plt count > 1 million

63
Q

Treatment for portal vein thrombosis

A

Heparin infusion acutely, then 6 months warfarin therapy.

64
Q

Do patients with essential thrombocythemia and polycythemia vera benefit from early splenectomy?

A

No, splenectomy offers little benefit for these diseases until myelofibrosis has developed.

65
Q

Preoperative management in patients undergoing planned splenectomy

A

Vaccinations 2 weeks preop

66
Q

What are the vaccinations indicated preop for planned splenectomy?

A

Vaccinations against encapsulated organisms: haemophilus influenza b, polyvalent Pneumococcus, Meningococcus

67
Q

What are relative contraindications to splenectomy?

A

Coagulopathy and portal hypertension

68
Q

Size limit of spleen for laparoscopic splenectomy

A

Should be less than 20-25cm in length.

69
Q

During splenectomy for ITP, when should platelets be infused?

A

After the splenic artery is doubly ligated.

70
Q

At reexploration for bleeding post splenectomy, what is the most common source identified?

A

Bleeding along the diaphragmatic portion of the splenic bed.

71
Q

Most common location for accessory spleen?

A

Splenic hilum

72
Q

Incidence of accessory spleens?

A

30%

73
Q

Incidence of OPSS (overwhelming postsplenectomy sepsis) in general population?

A

1-4%

74
Q

Incidence of OPSS in patients less than 5 with a history of hematologic disorders?

A

Approaches 12-15% in some reports

75
Q

Mortality associated with OPSS

A

50%

Cameron's Esophagus and Spleen (13 decks)

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