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Splenectomy for haematologic disorders
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== '''Indications for splenectomy in general''' == * Symptomatic splenomegaly ** Abdo pain ** Early satiety ** Weight loss ** Abdo distension * Cytopaenia * Unexplained splenomegaly - consider splenectomy == '''Relevant disorders''' == === '''Autoimmune and idiopathic disorders''' === ==== '''Immune thrombocytopaenia (ITP) -''' previously known as idiopathic thrombocytopaenic purpura ==== *** Most common haematological indication for resection *** ''Pathophysiology:'' Platelet destruction secondary to platelet autoantibodies. Production of IgG directed towards platelet glycoproteins increases destruction by reticuloendothelial system of spleen. *** ''Diagnosis:'' Thrombocytopaenia <100, despite normal bone marrow and the absence of other causes of thrombocytopaenia that could be responsible. **** Diagnosis of exclusion - consider HIV, SLE, APS, HCV, lymphoproliferative disorders, drugs (cocaine, gold, certain antibiotics, antihypertensives, anti-inflammatories, heparin, quinidine, abciximab), H. pylori infection **** Primary ITP when there is no clear aetiology. Newly-diagnosed if <3 months, persistent if 3-12 months, and chronic >12 months. **** Secondary ITP with a known cause. *** ''Presentation:'' Usually asymptomatic until platelets <30. 'platelet-type bleeding' is bruising, purpura, petechiae, bleeding from oral mucosa, epistaxis, menorrhagia, and GIT bleeding seen rarely. Intra-cerebral haemorrhage occurs in 1%. **** Does not present with splenomegaly, just hypersplenism *** ''Treatment'' **** Observation initially, especially in children, or if platelets >30 in adults **** First-line: Corticosteroids (1-2mg/kg prednisolone daily for 2-4 weeks followed by taper) if persistent thrombocytopaenia despite observation or platelets <30. 25% will have a complete response. **** Second-line: rituximab and thrombopoietin receptor antagonists. **** Platelets only given with severe haemorrhage. **** Splenectomy ***** Indications: ****** Low platelets <10 after 6-8 weeks of steroid therapy ****** Require toxic doses of steroids to achieve remission ****** Thrombocytopaenia refractory to medical treatment ****** Pregnant women in second trimester, with platelets <10 if asymptomatic or <30 if symptomatic, after medical treatment ***** NOT necessary when: ****** Platelets >50 ****** ITP >6 months ****** No bleeding symptoms ****** Not engaged in high-risk activities ***** Consider IVIg in preparation for OT. 1g/kg per day for two days. Usually increases platelet count within 3 days, and increases efficacy of platelet transfusion. ***** If platelets are required, give them after splenic artery has been ligated ideally ***** Splenectomy can generally be performed safely with minimal bleeding risk even in patients with platelets <10 ***** Outcomes ****** 75-85% permanent cure ****** Platelets normally improve within 10 days - likely to be durable if platelets >150 by day 3 or >500 by day 10 ***** Relapse ****** Evaluate for missed accessory spleen - can use radionuclide imaging ****** Remove it, if safe to do so ==== '''Thrombotic thrombocytopaenic purpura (TTP)''' ==== *** Deficiency of ADAMS13 protein leads to increased platelet aggregation and subsequent microvascular thrombosis *** Often precipitated by factors (chemotherapy agents, quinine, cyclosporine, clopidogrel, ticlopidine, haematopoietic stem cell transplantation, or pregnancy) *** Clinically: microangiopathic haemolytic anaemia, severe thrombocytopaenia, fever, neurologic complications, renal failure *** Initial treatment with plasmapheresis *** Splenectomy reserved for refractory thrombocytopaenia or frequent relapses ==== '''Autoimmune haemolytic anaemia''' ==== *** Autoantibodies are formed and directed against RBC antigens *** Warm **** Initially treated with corticosteroids **** Splenectomy indications ***** Fail to achieve remission in 3 weeks of steroids ***** Hb levels cannot be maintained with low-dose steroids **** 50% of patients will still require low-dose steroids to maintain Hb concentrations after splenectomy *** Cold **** Haemolysis occurs at low temperatures **** Usually caused by an infectious process such as EBV **** Steroids not usually effective **** Splenectomy is NOT indicated - RBCs are destroyed in the liver === '''Congenital disorders of the blood''' === ==== '''Hereditary spherocytosis''' ==== *** ''Pathophysiology:'' defect in RBC membrane. Usually autosomal dominant. Results in small, spherical RBCs that can't deform to fit through capillaries. *** Most common congenital anaemia *** Presentation **** Most commonly a moderate haemolytic anaemia, and sometimes with jaundice, folate deficiency, and splenomegaly **** Severe - anaemia, jaundice, splenomegaly, cholelithiasis with pigmented gallstones **** Spherocytes on blood smear, other signs of haemolysis *** Splenectomy is curative for almost all patients, but note that it doesn't fix the underlying problem **** Indicated if growth retardation, skeletal changes, symptomatic haemolytic disease, anaemia-induced organ dysfunction, leg ulcers, development of extramedullary haematopoietic tumours **** Controversial whether patients with mild disease should have splenectomy *** Preoperative USS should be performed, and cholecystectomy should be performed at same time as splenectomy if they have gallstones *** Delay surgery until after age 5 to prevent overwhelming post-splenectomy infection ==== '''Hereditary elliptocytosis''' ==== *** Rare disorder resulting from mutation of RBC membrane skeleton proteins *** Wide variety of clinical presentations *** Can lead to severe haemolysis *** Splenectomy indicated for those with symptomatic anaemia and is curative ==== '''Hereditary pyropoikilocytosis''' ==== *** Subtype of hereditary elliptocytosis *** Usually seen as anaemia and jaundice in newborns *** Splenectomy is curative ==== '''Hereditary stomacytosis and xerocytosis''' ==== *** Rare haemolytic anaemias *** In severe cases, consider splenectomy, need strong indication as complications common ==== '''Thalassaemia''' ==== *** Autosomal dominant inherited haematologic disorders caused by a defect in synthesis in one or more of the haemoglobin chains, with subsequent RBC destruction *** Heterozygous (thalassaemia minor) forms are usually asymptomatic, with microcytosis and mild anaemia *** Homozygous is much more severe - asymptomatic until 6 months old because of fetal haemoglobin, then become severely unwell **** Treatment is periodic lifelong blood transfusions and iron chelation therapy **** Splenectomy for increased blood transfusion requirements in setting of hypersplenism - some say >180-200ml/kg/year of pRBC should be threshold for splenectomy **** Massive splenomegaly is rare, but can be treated with splenectomy if interfering with quality of life **** 25-60% reduction in transfusion requirements can be expected after splenectomy **** Delay until after age 5 *** Can get acute splenic sequestration crisis in beta-thalassaemia - severe anaemia, splenomegaly and an acute bone marrow response. Same as below with sickle cell anaemia. Splenectomy to prevent repeat attack. ==== '''Sickle cell anaemia''' ==== *** ''Pathophysiology:'' Autosomal recessive haemoglobinopathy via point mutation in beta globin gene - RBCs have propensity to deform when exposed to low oxygen tension *** Splenectomy rarely indicated because of auto-infarction of the spleen - vasoocclusion and progressive infarction. Generally leads to atrophied spleen by adulthood. *** Indications **** Splenic abscess - generally as a complication of infarction. Fever, abdominal pain, tender splenomegaly. May require urgent splenectomy after stabilisation. **** Acute splenic sequestration - massive splenomegaly, acute exacerbation of anaemia, hypovolaemia. Initially treated by restoration of volume and RBC mass, but recurrence is common. Splenectomy can prevent further episodes. **** Developmental delay due to metabolic effects of disease ==== '''Pyruvate kinase deficiency''' ==== *** Defect in glycolytic pathway causes ATP deficiency. RBCs are less deformable and often destroyed in the spleen, leading to splenomegaly. *** Tends to cause chronic haemolysis *** Mild to severe anaemia accompanied by splenomegaly *** Splenectomy if severe haemolytic anaemia, or patients that require significant numbers of transfusions. Delay until after age 5. ==== '''G6PD deficiency''' ==== *** Typically seen in African, Middle-Eastern or Mediterranean ancestry *** Damage to red cell macromolecules by toxic oxygen products *** Tends to cause episodic haemolysis *** Haemolysis precipitated by acute infections, oxidant drugs, and fava beans *** Splenectomy is rarely, if ever, indicated === '''Neoplasms and myeloproliferative disorders''' === ==== '''Hodgkin's lymphoma''' ==== *** See separate topic under haematology *** Malignant neoplasm of lymphoreticular cell origin that usually affects young adults 10-30yo *** Primary treatment is CTX +/- radiation *** Historically, splenectomy was performed as part of a staging laparotomy that included lymph node sampling and liver biopsy - this is rarely done today as CT and PET are better *** Splenectomy can sometimes be useful in those who develop thrombocytopaenia or symptoms related to splenomegaly ==== '''NHL''' ==== *** See separate topic under haematology *** Most common type of lymphoma - diverse group of diseases *** Most common primary splenic neoplasms with 60-80% having splenic involvement **** Especially splenic marginal zone lymphoma - B cell lymphoma in older patients - splenectomy is diagnostic and therapeutic *** Indications for splenectomy **** Symptoms related to massive splenomegaly **** Cytopaenias resulting from splenic sequestration **** Requested by haematologists to obtain tissue for diagnosis or to guide therapy ==== '''Hairy cell leukaemia''' ==== *** See separate topic under haematology *** Rare *** Fatigue, LUQ pain, fever, infection, coagulopathy *** Commonly occurs in 5th decade *** Splenomegaly, pancytopaenia, neoplastic peripheral mononuclear cells, BM infiltration *** Splenectomy was historically important standard treatment, but now replaced by biologics *** Splenectomy now reserved for cases of incomplete response to first-line therapy, persistent splenomegaly in absence of BM involvement, atraumatic splenic rupture, and severe bleeding from thrombocytopaenia ==== '''Chronic lymphocytic leukaemia (CLL)''' ==== *** See separate topic under haematology *** B-cell leukaemia in which there is progressive accumulation of functionally incompetent lymphocytes *** Splenic infiltration is common, can lead to severe splenomegaly and substantial cytopaenias through hypersplenism *** Splenectomy can be indicated to relieve symptoms, or to relieve secondary ITP and AIHA ==== '''Chronic myelogenous leukaemia (CML)''' ==== *** See separate topic under haematology *** Disorder of abnormal proliferation and accumulation of granulocytes *** Characteristic Philadelphia chromosome *** Splenomegaly in 40% *** Acute blastic crisis can develop - severe splenomegaly and hypersplenism, leading to severe anaemia, bleeding complications and infection *** Splenectomy indicated for palliation of severe symptoms of splenomegaly/hypersplenism ==== '''Primary myelofibrosis''' ==== *** Chronic malignant haematologic disorder that results in hyperplasia of abnormal myeloid precursor cells leading to marrow fibrosis and extramedullary haematopoiesis in liver and spleen *** Splenectomy indicated in haemolysis requiring significant transfusions, thrombocytopaenia, symptomatic splenomegaly, recurrent splenic infarctions, hypercatabolic symptoms, and portal hypertension with refractory ascites and variceal haemorrhage *** Post-operative platelet-lowering agents can reduce thrombotic complications === '''Miscellaneous disorders''' === ==== '''Amyloidosis''' ==== *** Extracellular deposition of insoluble fibrillar proteins in tissues and organs *** Hepatosplenomegaly can occur in 25%, with severe splenomegaly in 10%, and they can develop functional splenic insufficiency *** Splenectomy indicated for signs and symptoms of splenomegaly, but does not alter ultimate course of disease *** Splenectomy may improve factor X levels which can drop if liver not functioning properly *** Pre-operative administration of factor VIIa is important ==== '''Gaucher's disease''' ==== *** Glycolipid storage disease - deposition of glococerebroside in reticuloendothelial system *** Leads to severe organomegaly, pulmonary infiltrates, BM infiltration *** Anaemia, thrombocytopaenia, osteopaenia, bone pain, osteonecrosis, massive hepatosplenomegaly *** Splenectomy indicated for severe and symptomatic splenomegaly and refractory cytopaenia - does not alter disease progression but removes chance of splenic rupture and can improve thrombocytopaenia *** Partial splenectomy advocated in some children to preserve some splenic function *** Splenectomy can actually increase risk of bone disease and lung/kidney function, so careful patient selection is essential ==== '''Felty's syndrome''' ==== *** RA, otherwise unexplained neutropaenia, and splenomegaly *** Treat with MTX or disease-modifying anti-rheumatic drugs *** GCSF may be useful pre-op *** Splenectomy indicated when medical treatment has failed, as evidenced by recurrent infections or severe neutropaenia. Results in 80% haematologic response rate. ==== '''Sarcoidosis''' ==== *** Non-caseating granulomatous disase *** 90% primary lung involvement, but can affect any organ *** Splenic involvement is mostly as part of multi-organ disease - occurs in 10% or so *** Treat with steroids, MTX *** Indications for splenectomy - symptomatic splenomegaly, intractable pain, exclusion of neoplastic process, hypersplenism *** Splenectomy does not alter disease course but can help with treatment of refractory hypercalcaemia ==== '''Idiopathic splenomegaly''' ==== *** Diagnostic and therapeutic *** About half of these patients have lymphoma == '''Pre-op considerations''' == * Assess size with CT/USS * Look at anatomic relationships, vascular anatomy, presence of accessory spleens, perisplenic lymphadenopathy, inflammation * Splenic artery embolisation can be helpful to reduce risk of blood loss, especially with Jehovah's witnesses (may cause severe pain and ischaemic complications) * Stress dose steroids * Give platelets only after ligating splenic artery * Cameron's says can do it safely even platelets <20 * Post-op vaccinations (give pre-op in elective setting) * Moderate splenomegaly: 11-20cm in greatest diameter * Massive splenomegaly: >20cm * Laparoscopic splenectomy should be possible even in massive splenomegaly (generally limited by size of retrieval device) - Cameron's says can do it up 25cm * Examine abdomen for splenunculi - most commonly gastrosplenic ligament and greater omentum [[Category:Spleen]]
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