non-traumatic indications for splenectomy...“nontraumatic indication for splenectomy are...

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Non-Traumatic Indications for Splenectomy

Christy Guth, M.D.

PGY-1

Vanderbilt Medical Center

Hematologic

ITP

TTP

Hereditary

spherocytosis

Hemoglobinopathy

Felty’s Syndrome

Neoplasms

Hodgkin’s

Non-Hodgkin’s

CML

CLL

Hemangioma

Hemangiosarcoma

Lymphangioma

Lymphangiosarcoma

Benign

Cysts

Abscess

Splenic Vein

Thrombosis

Presenter

Presentation Notes

“Nontraumatic indication for splenectomy are typlically hematologic conditions, which may affect red cells, white cells, or platelets. Splenomegaly, which causes pain and discomfort, but as a last resort.

Spleen Physiology

Hematopoiesis (until 5th month gestation)

Mechanical Filtration

Immune Function Tuftsin – stimulates macrophages and leukocytes Properdin – regulates complement activation Interferon – reduces viral replication

Presenter

Presentation Notes

“The spleen is a key organ for the removal of old and abnormal red cells, platelets, and neutrophils from the circulation. In addition, it plays an important immunological role and is essential in the production of IgM, tuftsin, properdin, and interferon.

Hematologic Indications

Platelet ITP TTP

RBCs Hereditary Spherocytosis Chronic autoimmune hemolytic anemias Hemoglobinopathies

WBCs Felty’s Syndrome

Immune Thrombocytopenic Purpura

Treatment Protocol

Glucocorticoids

IVIg/Plt Transfusion

Splenectomy

Platelet Antibodies Destruction Thombocytopenia

Presenter

Presentation Notes

IgG antibodies to platelet membrane glycoproteins Blood smear = thrombocytopenia, no giant platelets, normal RBCs, normal WBCs Splenectomy removes the source of antiplatelet Ig and phagocytic cells

Immune Thrombocytopenic Purpura

Children

Acute

Rarely require splenectomy Refractory > 1 year +/- Emergent for ICH

Adults

Chronic

Occasionally require splenectomy If plts < 10K after 6

wks < 30 K after 3 months Emergent for ICH

George, J, Woold, S, Raskob G, et al. Idiopathic Thrombocytopenic Purpura: A Practice Guideline Developed by Explicit Methods for the American Society of Hematology. Blood. 1996; 88(1): 3-40

Presenter

Presentation Notes

Two distinct clinical syndromes Children rarely require splenectomy, generally reserved for refractory cases that persist for > 1 year or earlier if uncontrolled hemorrhage occurred Up to 70% of children will recover within 3 weeks without specific treatment. Generally, if child has poor response to glucocorticoids, will have complete response to splenectomy and visa versa. “there are inadequate data to make evidence-based recommendations on the appropriate indications and timing for splenectomy.” Adults: splenectomy > 80% successful within 10 days If not, look for accessory spleen (10% of NR) Plt transfusion after splenic artery is ligated

Thrombotic Thrombocytopenic Purpura

ADAMTS13 antibodies Thombotic

microangiopathy

Presentation Thombocytopenia Red cell fragmentation Fever Neurological deficits Renal failure

Treatment Plasma exchange = 80% Splenectomy during

stable disease Kappers-Klunne M, Wijermans P, Fijnheer R, et al. Splenectomy for the treatment of thrombotic thrombocytopenic purpura. British Journal of Haemotology. 2005; 130: 768-776

Presenter

Presentation Notes

Metalloprotease that cleaves large multimers of von WIllebrand factor = extensive microscopic clots in small blood vessels = thrombotic microangiopathy (platelet thrombi in the microcirculation

Hereditary Spherocytosis

Spectrin Deficiency Spherocytes Osmotic Fragility

Direct Coomb’s (-)

Splenectomy >4yo

Laparoscopic approach recommended

Autoimmune Hemolytic Anemia

IgG antibodies against RBCs

Causes Idiopathic Leukemia/Lymphoma Systemic Lupus Erythematosus Drug induced (methyldopa, PCN, quinidine)

Direct Coomb’s (+)

Steroid therapy first, splenectomy if refractory

Sickle Cell Disease

Splenectomy for: Recurrent Splenic

Sequestration Crises Hypersplenism Splenic Abscess Massive Splenic Infarct

+/- Cholecystectomy

Thalassemia

Hypochromic anemias

Transfusion w/ chelation

Massive Splenomegaly

pRBCs >250cc/kg/year

Felty’s Syndrome

Rheumatoid Arthritis

Granulocytopenia

Splenomegaly

Splenectomy for severe granulocytopenia (<1000/mm3)

Neoplasms Hodgkin’s Lymphoma

historically used for staging, no longer routine

Non-Hodgkin’s Lymphoma symptomatic splenomegaly diagnosis/staging in isolated splenic disease

Hairy Cell Leukemia Symptomatic splenomegaly Severe cytopenia

CML symptomatic splenomegaly

CLL Symptomatic splenomegaly

Presenter

Presentation Notes

NHL is the most common primary splenic neoplasm Symptomatic splenomegaly = abdominal pain, fullness, early satiety

Vascular Neoplasms Angiosarcoma

Very aggressive Spontaneous splenic rupture and hemolytic anemia Palliative splenectomy

Benign – may cause symptomatic splenomegaly Hemangioma Lymphangioma Hamartoma

Presenter

Presentation Notes

Splenic hemanguima is a rare disorder, but remains the most common benign neoplasm of the spleen. <4cm unlikely to rupture. Angiosarcomas (exposure to thorium dioxide or monomeric vinyl chloride), can result in rupture, Splenectomy prior to rupture has been demonstrated to increase length of survival compared with splenectomy after rupture. But is rarely curative due to the aggressive and metastatic nature of the disease.

Metastasis

Most common: breast, lung, melanoma, ovary

Usually asymptomatic

Occasional symptomatic splenomegaly or spontaneous rupture

Parasitic Cyst

Parasitic Ecchinoccocus

granulosis Hydatid disease Do not rupture

Non-Parasitic Cyst

True Cyst Epithelial lined Epidermoid cysts Symptomatic,

bleeding, infection, rupture

Partial splenectomy

Pseudocyst No epithelial lining Post-traumatic >10cm Symptomatic

Splenic Abscess

Multiloculated

Not amenable to IR drain

Failed to resolve with IR drain and abx

Splenic Vein Thrombosis

Chronic pancreatitis

Sinistral Hypertension

Gastroesophgeal variceal bleeding

Splenectomy at the time of pancreas operation

Presenter

Presentation Notes

Chronic pancreatitis is the most common cause of SVT. Symptomatic SVT, if isolated thrombosis, ok for splenectomy at the time of pancreatic surgery (pancreaticojejunostomy 20-40% with CP get SVT, 3-72% of these have gastroesophageal variceal bleeding.

Splenectomy Vaccines

Pneumoccocus Booster every 5 years

H. influenzae Annual vaccine

Meningococcus

Presenter

Presentation Notes

Vaccines against encapsulated bacterial pathogens

Summary

Hematologic Indications Does not cure underlying disease Does correct underlying hematologic abnormalities Alleviates symptoms Used for staging and diagnosis

References

Kappers-Klunne et al. (2005) Splenectomy for the treatment of thrombotic thrombocytopenic purpura. British Journal of Haematology, 130, 768—776.

Wood et al. (2010) Predicting response to splenectomy in children with immune thrombocytopenic purpura. Journal of Pediatric Surgery, 45, 140—144.

George et al. (1996) Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology, 88, 2—40.

References Williams & Glazer (1993) Splenic cysts: changes in

diagnosis, treatment and aetiological concepts. Annals of the Royal College of Surgeons of England, 75, 87—89.

Smith (1960) The role of splenectomy in the management of thalassemia. Blood, 15, 197—211.

Bolton-Maggs (2011) Guidelines for the diagnosis and management of hereditary spherocytosis – 2011 update. British Journal of Haematology, 156, 37—49.

Alwabari et al (2009) Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease. Pediatric Surgery International, 25, 417—421.

non-traumatic indications for splenectomy...“nontraumatic indication for splenectomy are...

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