1. Splenectomy in Hematologic Disorders
Scott Nguyen
Team 4
6/11/04

2. Indications Idiopathic Thrombocytopenic Purpura (ITP)
Hereditary Spherocytosis
Chronic Autoimmune Hemolytic Anemia
Non Hodgkins Lymphoma
Hairy Cell Leukemia
Chronic Lymphocytic Lymphoma / Chronic Myelogenous Lymphoma

3. Chronic ITP Autoimmune disorder of Adults
Autoantibodies to platelet glycoproteins
Antibodies act as opsonins and accelerate platelet clearance by phagocytic cells
Also can bind to critical regions of the glycoproteins and impair function
F > M 3:1, ages 20-40

4. Chronic ITP Purpura, epistaxis, gingival bleeding
Rarely GI, GU, intracranial hemorrhage
Diagnosis – low platelet count, normal bone marrow, exclusion of other causes of thrombocytopenia
Drugs
Viral infections
Autoimmune diseases
Lymphoproliferative diseases

5. ITP - Management Assx disease w/ Plts > 50K – no tx needed
If < 50K, bleeding – tx indicated
Glucocorticoids – Prednisone 1mg/kg
2/3 respond initially
25% completely respond
Acute severe bleeding
Platelet transfusions
IVIG

6. Refractory ITP
Most respond to steroids, but >75% pts recurr after steroids tapered
Splenectomy – removes source of antiplatelet Ig, removes source of phagocytic cells
Indications –
Plts < 10K after 6 wks med tx
Plts < 30K, had insuffic response to med tx after 3mos
Emergent splenectomy in cases of intracranial bleeding
Platelet transfusions should only be given after splenic artery ligated to prevent destruction

7. Splenectomy for ITP 65-80% successful
Usually platelet counts respond by 10 days
Age < 60, good inititial response to steroids are favorable factors
Laparoscopic splenectomy popular as spleen is usually small to normal sized

8. Unsuccessful Splenectomy
Missed accessory spleen in 10%
Can localize w/ Radionuclide imaging
Long term steroid therapy
Azathioprine or cyclophosphamide

9. Hereditary Spherocytosis
Autosomal dominant deficiency of spectrin, red cell cytoskeletal protein - maintains osmotic stability
Membrane abnormality results in red cells which are small, spherical, and rigid
Spherocytes more susceptible to becoming trapped in spleen and destroyed

10. Hereditary Spherocytosis

11. Hereditary Spherocytosis
Clinical presentation – anemia, jaundice, cholelithiasis, splenomegaly
Diagnosis –
Family history of anemia
spherocytes on peripheral smear
Increased reticulocyte count
Increased osmotic fragility
Negative Coombs test

12. Treatment Splenectomy decreases rate of hemolysis
If diagnosed in childhood, splenectomy should wait until after 4yo to preserve immunologic function of spleen
Cholecystectomy can be performed at same operation if documented gallstones

13. Autoimmune Hemolytic Anemia
Conditions in which autoantibodies against pt’s own red cells are formed (IgG)
Abnormal destruction
“marked” RBCs are prematurely destroyed by phagocytic cells
Complement activated on cell membranes – resulting in lysis

14. Causes
Idiopathic
Lymphoproliferative diseases – CLL, NHL, Hodgkins disease
Systemic Lupus Erythematosus or other Collagen Vascular diseases
Postviral infections
Drug induced (methyldopa, pcn, quinidine)

15. Autoimmune hemolytic anemia
More in adults, F > M
Moderate to severe anemia, high reticulocytes
Spherocytosis on blood smears
Splenomegaly
Direct Coomb test positive

16. Direct Coombs test

17. Treatment Make sure not drug related Steroids –
75% respond, 50% relapse
Splenectomy for those who fail steroid therapy
Refractory cases – azathioprine and cyclophosphamide, other immunosuppressive drugs

18. Hodgkins Lymphoma
Splenectomy routinely performed during staging laparotomy
Confirms disease below the diaphragm (upstages II -> III)
Radiation tx -> Chemotx
Historically performed often
Advances in imaging – CT, lymphangiography, PET – much improved nonoperative staging

19. Hairy Cell Leukemia 2% adult leukemias
Hairy Cells – neoplastic B lymphocytes w/ cell membrane ruffling
Found in peripheral blood and bone marrow
Usually in elderly men
Symptoms d/t pancytopenia from hypersplenism and infiltration of bone marrow

20. Hairy Cell Leukemia

21. Hairy Cell Leukemia
Massive splenomegaly causing hypersplenism – rapidly and prematurely destroy all blood cells
Symptomatic anemia
Infectious complications from neutropenia
Bleeding complications from thrombocytopenia
Increased risk of second malignancy

22. Treatment Alpha interferon, purine analogs
Splenectomy in refractory cases
40% pts get normalization of blood counts after
Usually response lasts > 10 yrs
50% pts require no further tx

23. Splenectomy CML and CLL Pyruvate Kinase deficiency
Hemoglobinopathies – sickle cell, thalassemia