History

Fact Explanation
Abdominal discomfort Spleen is mainly responsible for the blood cell destruction, lymphocyte production and blood storage in the body. [1] In hypersplenism, there is an overactive spleen with increased blood cell destruction and storage. Hypersplenism causes cytopenia, (reduction of the cell counts). In pancytopenia there is a reduction in all 3 cell lines: red blood cells, white blood cells and platelets or cytopenia affecting only one or two cell lines. Neutropenia is less common than anaemia and thrombocytopenia. Splenomegaly causes discomfort to the person specially over the left upper part of the abdomen.
Fever and other features of infections White blood cells are needed for defence against the infections. Neutropenia causes increased vulnerability for the infections which may be very severe and needs antibiotics to settle. [1]
Shortness of breath, fatigue and lethargy Reduction in the red blood cells causes anaemia. Lack of energy due to low oxygen in the blood in anaemia causes fatigability and lethargy. [3]
Bleeding manifestations:gum and nose bleeding, menorrhagia, excessive bleeding from the cuts etc Due to the low platelet counts [2] , they develop bleeding manifestations of the skin and mucous membranes.
Loss of appetite, loss of weight, low grade fever There are 2 types of hypersplenism:primary and secondary. [2] If there is an unknown pathology with direct involvement of the spleen, it is primary whereas in secondary hypersplenism it is due to a known underlying pathology. Primary causes are idiopathic thrombocytopenic purpura, congenital hemolytic anaemia and primary splenic neutropenia or pancytopenia. Secondary causes are either directly involving the splenic tissue as in the case of lymphoma, leukaemia, metastatic carcinoma, chronic/acute infections or indirectly affecting spleen as in collagen diseases, Felty's syndrome, Gaucher's disease and cirrhosis.
Epigastric pain rediating directly to the back Pancreatitis [4] can cause splenic vein thrombosis, leading to hypersplenism.
History of measles or chickenpox These viral infections can trigger thrombocytopenic purpura. [2]
Alcoholism, haematomesis Alcoholism can cause cirrhosis that inturn causes congestive splenomegaly. [2]
References
  1. SCHATKEN RV. Hypersplenism: A Review and Case Report Ann Surg [online] 1952 Apr, 135(4):536-542 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1802490
  2. DUCKETT JW. Splenectomy in Treatment of Secondary Hypersplenism Ann Surg [online] 1963 May, 157(5):737-746 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1466526
  3. KNOTTNERUS JA, KNIPSCHILD PG, WERSCH JW, SIJSTERMANNS AH. Unexplained Fatigue and Hemoglobin: A Primary Care Study Can Fam Physician [online] 1986 Aug:1601-1604 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2327457
  4. CHURCH NI, PEREIRA SP, CHURCHILL D, CAIRNS S, HATFIELD AR, WEBSTER GJ. Chronic pancreatitis: diagnosis and management of complications Gut [online] 2007 Sep, 56(9):1189-1190 [viewed 15 September 2014] Available from: doi:10.1136/gut.2007.132498

Examination

Fact Explanation
Splenomegally [1] Splenomegaly [1] is an essential component in hypersplenism. However there are instances such as essential thrombocytopenic purpura where the spleen may be not enlarged.
Pallor Due to the anaemia. [4]
Exertional dyspnea Due to the anaemia. [3]
Febrile Neutropenia [4] causes increased vulnerability for infections.
Purpura and bruising Idiopathic thrombocytopenic purpura is a primary cause for hypersplenism. [2]
Lymphadenopathy Secondary causes for hypersplenism such as lymphoma, leukaemia, metastatic carcinoma, chronic/acute infections [6] can cause lymphadenopathy. [2]
Jaundice May be secondary to liver disease. [5]
Ankle edema, peripheral stigmata of chronic liver disease:jaundice, telangiectasias, gynecomastia, caput medusa, and ascites Cirrhosis may be a cause for the splenomegaly. [2]
Features of rheumatoid arthritis : Tenderness and swelling of the metacarpophalangeal interphalangeal joints and metatarsophalangeal joints, ulnar deviation of the wrist, Z thumb deformity, buttonhole deformity Felty's syndrome [4] , is a complication of rheumatoid arthritis. Components of Felty's syndrome are splenomegaly with neutropenia.
References
  1. SCHATKEN RV. Hypersplenism: A Review and Case Report Ann Surg [online] 1952 Apr, 135(4):536-542 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1802490
  2. DUCKETT JW. Splenectomy in Treatment of Secondary Hypersplenism Ann Surg [online] 1963 May, 157(5):737-746 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1466526
  3. MOTTRAM PM, MARWICK TH. Assessment of diastolic function: what the general cardiologist needs to know Heart [online] 2005 May, 91(5):681-695 [viewed 10 September 2014] Available from: doi:10.1136/hrt.2003.029413
  4. SANDUSKY WR, RUDOLF LE, LEAVELL BS. Splenectomy for control of neutropenia in Felty's syndrome. Ann Surg [online] 1968 May, 167(5):744-751 [viewed 10 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1387126
  5. CAMPISI DJ, MCELROY CR, CRILEY JM, GILKEY F. Jaundice, hepatosplenomegaly and liver failure in a 60-year-old woman. West J Med [online] 1981 Jan, 134(1):27-33 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1272445
  6. FARUKHI SN, FOX JC. The role of ultrasound in the management and diagnosis of infectious mononucleosis Crit Ultrasound J [online] , 6(1):4 [viewed 15 September 2014] Available from: doi:10.1186/2036-7902-6-4

Differential Diagnoses

Fact Explanation
Hereditory spherocytosis and hemolytic anaemia Hereditary spherocytosis is an autosomal dominant red blood cell membrane defect with spherical red blood cells [6] which undergoes extravascular haemolysis. It causes splenomegaly. There will be spherocytes in the blood film and increased osmotic fragility apart from the other findings of haemolytic anaemia. There are 2 types of hemolytic anaemia: acquired and hereditary. There are 2 types of autoimmune haemolytic anaemia:warm and cold. Warm type is IgG mediated at temperature less than 4 centigrades that differ from cold type as it is IgM mediated at temperature 37 centigrades. This causes extravascular haemolysis. Direct and indirect coomb’s tests are positive and there will be spherocytes in the blood film.
Haemoglobinopathies Sickle cell anemia, alpha thalassemia and beta thalassemia are the haemoglobinopathies that can present with splenomegaly. Thalassemia [3] causes ineffective erythropoiesis, due to excess production of free alpha (α) globin chains. Thalassemia [8] patients have anemia that requires chronic blood transfusions and chelation therapies to prevent iron overload. Beta-thalassemia intermedia, is relatively a mild disease that does not require chronic blood transfusions. On examination they will have thalassemic facies maxillary hyperplasia, splenomegaly etc. These patients will also have the microcytic hypochromic anaemia with normal red cell distribution width.
Lymphoma, leukaemia These are lymphoproliferative disorders that may present with cytopenia either due to disease or treatment. [7]
Rheumatoid arthritis Felty's syndrome is a complication of rheumatoid arthritis comprised of splenomegaly and neutropenia. [5]
Malaria Malaria can cause splenomegaly. They usually present with every third or fourth day fever with other complications such as anaemia, jaundice, splenomegally, hepatomegaly, pulmonary and intestinal complications and cerebral malaria. [4] Thick and thin blood films are done to check for malaria parasites.
Gaucher disease This is a progressive lysosomal storage disorder due to the deficiency of glucocerebrosidase, leading to intralysosomal storage of glucosylceramide and dysfunction in multiple organ systems. [2] There rae main 3 types of the disease where type 1 is the most common type. Splenomegaly which may be moderate to massive, hepatomegaly, hepatic fibrosis occur commonly. Other features may be bone pain, pathological fractures, diarrhea, weight loss, tremor, supranuclear gaze palsy, corneal opacity, and cardiovascular calcification etc. Diagnosis is made by DNA testing or enzyme assays to demonstrate the deficiency. [1] Enzyme replacement is the available treatment option.
References
  1. BOHRA V, NAIR V. Gaucher's disease Indian J Endocrinol Metab [online] 2011, 15(3):182-186 [viewed 08 August 2014] Available from: doi:10.4103/2230-8210.83402
  2. FICICIOGLU C. Review of miglustat for clinical management in Gaucher disease type 1 Ther Clin Risk Manag [online] 2008 Apr, 4(2):425-431 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2504062
  3. RECHAVI G, RIVELLA S. Regulation of Iron Absorption in Hemoglobinopathies Curr Mol Med [online] 2008 Nov, 8(7):646-662 [viewed 27 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3722362
  4. FLEGEL KM. Symptoms and signs of malaria. Can Med Assoc J [online] 1976 Sep 4, 115(5):409-410 [viewed 06 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1878692
  5. SANDUSKY WR, RUDOLF LE, LEAVELL BS. Splenectomy for control of neutropenia in Felty's syndrome. Ann Surg [online] 1968 May, 167(5):744-751 [viewed 10 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1387126
  6. CROOM RD 3RD, MCMILLAN CW, ORRINGER EP, SHELDON GF. Hereditary spherocytosis. Recent experience and current concepts of pathophysiology. Ann Surg [online] 1986 Jan, 203(1):34-39 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1251036
  7. VAKKALANKA B, LINK BK. Neutropenia and Neutropenic Complications in ABVD Chemotherapy for Hodgkin Lymphoma Adv Hematol [online] 2011:656013 [viewed 15 September 2014] Available from: doi:10.1155/2011/656013
  8. SCRIVER CR, BARDANIS M, CARTIER L, CLOW CL, LANCASTER GA, OSTROWSKY JT. Beta-thalassemia disease prevention: genetic medicine applied. Am J Hum Genet [online] 1984 Sep, 36(5):1024-1038 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1684522

Investigations - for Diagnosis

Fact Explanation
Full blood count Hypersplenism caused cytopenia, (reduction of the cell counts) It may be pancytopenia where there is a reduction in all 3 cell lines: red blood cells, white blood cells and platelets or cytopenia affecting only one or two cell lines. Neutropenia[2] is relatively less common that anaemia and thrombocytopenia. [1]
Haemoglobin level May be reduced in anaemia. [1]
Bone marrow examination As a compensatory mechanism for the hypersplenism, bone marrow produces excess blood cells and their progenitors giving rise to hyperplastic marrow. [1]
Epinephrine test Demonstration of splenic over activity by epinephrine test aids the diagnosis of hypersplenism. [1] Two complete blood counts are done during a period of to evaluate the red cells, haemoglobin, white cells and platelets. Next 0.5 cc of 1/1000 epinephrine is given subcutaneously. Then every 10 minutes for 1 hour, complete blood counts are done. It does not produce a positive test in essential thrombocytopenic purpura and congenital haemolytic icterus.
References
  1. SCHATKEN RV. Hypersplenism: A Review and Case Report Ann Surg [online] 1952 Apr, 135(4):536-542 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1802490
  2. SANDUSKY WR, RUDOLF LE, LEAVELL BS. Splenectomy for control of neutropenia in Felty's syndrome. Ann Surg [online] 1968 May, 167(5):744-751 [viewed 10 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1387126

Investigations - Fitness for Management

Fact Explanation
Full blood count Neutropenia causes increased vulnerability for the infections which may be very severe and needs antibiotics to settle. [1]
References
  1. SCHATKEN RV. Hypersplenism: A Review and Case Report Ann Surg [online] 1952 Apr, 135(4):536-542 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1802490

Investigations - Followup

Fact Explanation
Full blood count It is important to follow up the patient for the blood counts and the level of haemoglobin as there is cytopenia. [1]
References
  1. SCHATKEN RV. Hypersplenism: A Review and Case Report Ann Surg [online] 1952 Apr, 135(4):536-542 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1802490

Investigations - Screening/Staging

Fact Explanation
Bone marrow biopsy Conditions like aplastic anaemia, hypoplastic anaemia and myelofibrosis can also produce pancytopenia although the the criteria required for the diagnosis of hypersplenism may be absent. [1]
Ultrasound scan May be needed to evaluate splenomegaly specially if there is a doubt about the examination findings. [2] It is also important to visualize the splenic vessels. liver pathologies etc.
References
  1. DUCKETT JW. Splenectomy in Treatment of Secondary Hypersplenism Ann Surg [online] 1963 May, 157(5):737-746 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1466526
  2. FARUKHI SN, FOX JC. The role of ultrasound in the management and diagnosis of infectious mononucleosis Crit Ultrasound J [online] , 6(1):4 [viewed 15 September 2014] Available from: doi:10.1186/2036-7902-6-4

Management - General Measures

Fact Explanation
Supportive management Severe refractory anaemia requires blood transfusion. If there are infections due to low platelets, most of the time they require antibiotics. [1]
Platelet transfusions Patients with platelet counts below 50 × 109/L need prophylactic transfusions to increase platelet counts before procedures. [2]
Treat the underlying disease Secondary hypersplenism may be due to various causes as mentioned above and need specific treatment according to the cause. [3]
Preparing the patient for the splenectomy Due to the risk of postsplenectomy sepsis surgery [1] is usually delayed till 6-7 years of age. After the surgery they are vulnerable to infections with encapsulated organisms such as Pneumococcus species, Meningococcus species, and Haemophilus influenzae. [4,5] Therefore they should be vaccinated for pneumococci, meningococci and H.influenzae before the surgery. It is better to give these vaccines at least three weeks prior to removal of the spleen to optimize the immune response. Hemophilus influenza vaccine is given to patients > 2 years of age and at the time of routine vaccination for younger children. Quadrivalent meningococcal vaccine is also given for those who are above the age of 2. Patients should be advised to avoid crowded places or any other source of infections and to seek prompt medical evaluation for all febrile illnesses. Post operative penicillin prophylaxis is then started.
References
  1. SCHATKEN RV. Hypersplenism: A Review and Case Report Ann Surg [online] 1952 Apr, 135(4):536-542 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1802490
  2. HAYASHI H, BEPPU T, SHIRABE K, MAEHARA Y, BABA H. Management of thrombocytopenia due to liver cirrhosis: A review World J Gastroenterol [online] 2014 Mar 14, 20(10):2595-2605 [viewed 08 August 2014] Available from: doi:10.3748/wjg.v20.i10.2595
  3. FARUKHI SN, FOX JC. The role of ultrasound in the management and diagnosis of infectious mononucleosis Crit Ultrasound J [online] , 6(1):4 [viewed 15 September 2014] Available from: doi:10.1186/2036-7902-6-4
  4. HALLER JA JR, JONES EL. Effect of splenectomy on immunity and resistance to major infections in early childhood: clinical and experimental study. Ann Surg [online] 1966 Jun, 163(6):902-908 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1477047
  5. Overwhelming infection after splenectomy. Br Med J [online] 1967 Sep 23, 3(5568):752-753 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1843269

Management - Specific Treatments

Fact Explanation
Spleenctomy This is the curative option for the hypersplenism in most occasions. [1] In secondary hypersplenism though the splenectomy improves the cytopenic state, it has no effect on the underlying condition. Splenectomy is indicated in hereditary spherocytosis, other hemolytic anaemias and idiopathic thrombocytopenic purpura , where steroids are not successful, aplastic anaemia and myeloproliferative disorders, congestive splenomegaly with hypersplenism, lymphomatous and granulomatous disease especially where the other treatment modalities have failed. [2]
References
  1. SCHATKEN RV. Hypersplenism: A Review and Case Report Ann Surg [online] 1952 Apr, 135(4):536-542 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1802490
  2. SANDUSKY WR, LEAVELL BS, BENJAMIN BI. Splenectomy: Indications and Results in Hematologic Disorders Ann Surg [online] 1964 May, 159(5):695-709 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1408650