Hypersplenism

Hematology

Clinicals - History

Fact Explanation
Abdominal discomfort Spleen is mainly responsible for the blood cell destruction, lymphocyte production and blood storage in the body. 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. Abdominal discomfort
Spleen is mainly responsible for the blood cell destruction, lymphocyte production and blood storage in the body. 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. 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.
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. 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.
Bleeding manifestations:gum and nose bleeding, menorrhagia, excessive bleeding from the cuts etc Due to the low platelet counts , they develop bleeding manifestations of the skin and mucous membranes. Bleeding manifestations:gum and nose bleeding, menorrhagia, excessive bleeding from the cuts etc
Due to the low platelet counts , 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. 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. Loss of appetite, loss of weight, low grade fever
There are 2 types of hypersplenism:primary and secondary. 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 can cause splenic vein thrombosis, leading to hypersplenism. Epigastric pain rediating directly to the back
Pancreatitis can cause splenic vein thrombosis, leading to hypersplenism.
History of measles or chickenpox These viral infections can trigger thrombocytopenic purpura. History of measles or chickenpox
These viral infections can trigger thrombocytopenic purpura.
Alcoholism, haematomesis Alcoholism can cause cirrhosis that inturn causes congestive splenomegaly. Alcoholism, haematomesis
Alcoholism can cause cirrhosis that inturn causes congestive splenomegaly.

Clinicals - Examination

Fact Explanation
Splenomegally Splenomegaly is an essential component in hypersplenism. However there are instances such as essential thrombocytopenic purpura where the spleen may be not enlarged. Splenomegally
Splenomegaly 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. Pallor
Due to the anaemia.
Exertional dyspnea Due to the anaemia. Exertional dyspnea
Due to the anaemia.
Febrile Neutropenia causes increased vulnerability for infections. Febrile
Neutropenia causes increased vulnerability for infections.
Purpura and bruising Idiopathic thrombocytopenic purpura is a primary cause for hypersplenism. Purpura and bruising
Idiopathic thrombocytopenic purpura is a primary cause for hypersplenism.
Lymphadenopathy Secondary causes for hypersplenism such as lymphoma, leukaemia, metastatic carcinoma, chronic/acute infections can cause lymphadenopathy. Lymphadenopathy
Secondary causes for hypersplenism such as lymphoma, leukaemia, metastatic carcinoma, chronic/acute infections can cause lymphadenopathy.
Jaundice May be secondary to liver disease. Jaundice
May be secondary to liver disease.
Ankle edema, peripheral stigmata of chronic liver disease:jaundice, telangiectasias, gynecomastia, caput medusa, and ascites Cirrhosis may be a cause for the splenomegaly. Ankle edema, peripheral stigmata of chronic liver disease:jaundice, telangiectasias, gynecomastia, caput medusa, and ascites
Cirrhosis may be a cause for the splenomegaly.
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 , is a complication of rheumatoid arthritis. Components of Felty's syndrome are splenomegaly with neutropenia. 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 , is a complication of rheumatoid arthritis. Components of Felty's syndrome are splenomegaly with neutropenia.

Investigations - 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 is relatively less common that anaemia and thrombocytopenia. 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 is relatively less common that anaemia and thrombocytopenia.
Haemoglobin level May be reduced in anaemia. Haemoglobin level
May be reduced in anaemia.
Bone marrow examination As a compensatory mechanism for the hypersplenism, bone marrow produces excess blood cells and their progenitors giving rise to hyperplastic marrow. Bone marrow examination
As a compensatory mechanism for the hypersplenism, bone marrow produces excess blood cells and their progenitors giving rise to hyperplastic marrow.
Epinephrine test Demonstration of splenic over activity by epinephrine test aids the diagnosis of hypersplenism. 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. Epinephrine test
Demonstration of splenic over activity by epinephrine test aids the diagnosis of hypersplenism. 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.

Investigations - Management

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. Full blood count
It is important to follow up the patient for the blood counts and the level of haemoglobin as there is cytopenia.
Full blood count Neutropenia causes increased vulnerability for the infections which may be very severe and needs antibiotics to settle. Full blood count
Neutropenia causes increased vulnerability for the infections which may be very severe and needs antibiotics to settle.
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. 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.
Ultrasound scan May be needed to evaluate splenomegaly specially if there is a doubt about the examination findings. It is also important to visualize the splenic vessels. liver pathologies etc. Ultrasound scan
May be needed to evaluate splenomegaly specially if there is a doubt about the examination findings. It is also important to visualize the splenic vessels. liver pathologies etc.

Management - Supportive

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. Supportive management
Severe refractory anaemia requires blood transfusion. If there are infections due to low platelets, most of the time they require antibiotics.
Platelet transfusions Patients with platelet counts below 50 × 109/L need prophylactic transfusions to increase platelet counts before procedures. Platelet transfusions
Patients with platelet counts below 50 × 109/L need prophylactic transfusions to increase platelet counts before procedures.
Treat the underlying disease Secondary hypersplenism may be due to various causes as mentioned above and need specific treatment according to the cause. Treat the underlying disease
Secondary hypersplenism may be due to various causes as mentioned above and need specific treatment according to the cause.
Preparing the patient for the splenectomy Due to the risk of postsplenectomy sepsis surgery 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. 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. Preparing the patient for the splenectomy
Due to the risk of postsplenectomy sepsis surgery 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. 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.

Management - Specific

Fact Explanation
Spleenctomy This is the curative option for the hypersplenism in most occasions. 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. Spleenctomy
This is the curative option for the hypersplenism in most occasions. 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.

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