Splenomegaly

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2], Gichoya Judy Wawira [3],

Synonyms and keywords: Spleen enlargement; enlarged spleen; spleen swelling

Overview

Pathophysiology

Causes

The causes of splenomegaly vary widely and range from increased splenic function (such as hemoglobinopathies), splenic congestion due to abnormal blood flow (such as venous obstruction), and splenic infiltration (such as storage disorders and malignancies).

Causes by Pathogenesis

Mechanism Pathogenesis Example
Increased function Removal of defective RBCs
Immune hyperplasia Response to infection (viral, bacterial, fungal, parasitic)
Disordered immunoregulation
Drug reactions
Extramedullary hematopoiesis
Abnormal blood flow Organ Failure
Vascular
Infections
Infiltration Metabolic diseases
Benign and malignant infiltrations

Differentiating Splenomegaly from Other Diseases

Category Disease Etiology Clinical manifestations Para−clinical findings Gold standard Associated findings
Demography History Symptoms Signs Lab Findings Imaging
Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other CBC Iron LFT PT/PTT BUN/Cr UA Histopathology
WBC Hb Plt
Congestive Cirrhosis[1] Any age, more common in elderly + ↑↓ + + Nl + + Nl Liver biopsy
Heart failure[2] Elderly + ↑↓ ± + Nl Nl NA Echocardiography
Thrombosis of portal, hepatic, or splenic veins[3] Any age + + + + Nl ± + Nl NA Doppler ultrasonography
Malignancy Lymphoma[4] Any age, most common malignant splenic tumor + + + + Nl Nl + High ferritin Nl Lymph node biopsy
Leukemia[5] Elderly, male>female + + + + Nl Nl ± + High ferritin Nl
  • Leukemic blasts
  • Leukoerythroblastic features
  • Giant platelets
NA Bone marrow examination + clinical manifestation
Polycythemia Vera[6] Mean age >60 years old
  • Positive family history
+ Nl + Nl Nl Nl to ↑ Nl to ↑ High ferritin Nl Nl Nl Nl
  • Elevated normochromic, normocytic RBCs
  • Thrombocytosis
  • Rarely immature cells
  • Leukoerythroblastic picture
NA WHO criteria for PV
Disease Etiology Demography History Fever Fatigue Weight loss Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Multiple myeloma[7] Any age, more common in adults + + + + Nl Nl + High ferritin Nl
  • Blast cells
Bone marrow examination
Essential thrombocythemia[8]
  • Acquired mutation of JAK2, CALR, or MPL
Mean age >60 years old, female > male ± + Nl + + Nl Nl ↑↑ Nl Nl Nl Nl Nl Bone marrow biopsy
Primary myelofibrosis[9] Mean age >60 years old, male> female + Nl + + Nl Nl + ↑/↓ Low iron Nl Nl Bone marrow biopsy
Primary splenic tumors[10] Very rare
  • Positive family history
+ + + Nl Nl Low iron, low ferritin
  • Different histopathologic features depends on the type of tumor
Biopsy
Metastatic solid tumors[11] Any age, more in adults + + + + Nl ± ± Low iron, high ferritin
  • Blast cells
Biopsy
Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Infection Viral Hepatitis[12]
  • Any age
  • High risk behaviors
  • Close contact
+ + + + Nl Nl + + Nl NA Clinical manifestation + lab tests
Infectious mononucleosis[13]
  • More common in children and young adults
  • Close contact
+ + Nl + Nl Nl + Nl Nl Nl Nl Nl NA NA Clinical manifestation + heterophile antibody test
Cytomegalovirus[14]
  • More common in:
    • Immunocompromised patients
    • During pregnancy
  • Close contact
+ + Nl + Nl Nl + Nl Nl Nl Nl Nl NA NA ELISA
Bacterial Salmonella[15]
  • Any age
  • Eating contaminated meat, eggs, or milk
+ Nl + Nl
  • Diarrhea
  • Abdominal cramps
  • Vomiting
  • Dehydration
Nl Nl Nl Nl NA NA Stool test
  • Typhoid fever
  • Reactive arthritis
  • Irritable bowel syndrome
Brucella[16]
  • Brucella
  • Any age
  • Ingesting contaminated food such as unpasteurized milk products
  • Direct contact with an infected animal
  • Inhalation of aerosols
+ + Nl + Nl +
  • Headache
  • Vomiting
  • Diarrhea
  • Constipation
  • Arthralgia
  • Night sweats
  • Anorexia
  • Foul-smelling perspiration
Nl Nl Nl Nl NA NA Blood culture
  • Arthritis
  • Epididymo-orchitis
  • Spondylitis
  • Neurobrucellosis
  • Liver abscess formation
  • Endocarditis
Tuberculosis[17]
  • Mycobacterium tuberculosis
  • Any age
  • More common in developing countries and among immunocompromised patients
  • Close contact
  • HIV
+ + + Nl +
  • Chronic cough with blood-containing sputum
  • Night sweats
  • Anorexia
  • Chest pain
Nl Nl
  • Acid-fast bacillus
  • Cavity on chest x ray
Identifying M. tuberculosis in a clinical sample
  • Tuberculous meningitis
  • Urogenital tuberculosis
  • Pott disease
  • Miliary tuberculosis
Parasitic Malaria[18]
  • Plasmodium
  • Worldwide
  • More common in tropical and subtropical regions
  • Bitten by Anopheles mosquito
+ + Nl + + Nl + Nl Nl NA
Kala-azar[19]
  • Any age
  • Bitten by sandflies
+ + + + Nl + + Nl
  • Visualization of the characteristic amastigote in smears or tissue
NA Culture
  • Could be fatal
Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Inflammation Sarcoidosis[20]
  • Positive family history
+ + + + Nl Nl Nl Nl Nl Nl Nl NA Diagnosis of exclusion
Serum sickness[21]
  • Any age
  • Consumption of certain medications
+ + Nl + Nl ↓ or ↑

Eosinophilia

Nl Nl Nl NA Clinical manifestation + recent medication consumption
Systemic lupus erythematosus[22]
  • More common in young females
  • Positive family history
+ + + + Nl to ↓ Nl + + Clinical findings + laboratory studies
  • Multi organ damage
Felty syndrome[23] + + + + Nl to ↓ Nl + + Nl Nl Nl Nl Nl Clinical findings + laboratory studies
Infiltrative Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Gaucher disease[24]
  • Positive family history
- + + + Nl to ↓ Nl + + Nl Nl NA Enzyme testing
Niemann-Pick disease[25]
  • Positive family history
- + + + Nl to ↓ Nl + + Nl NA Genetic testing
Langerhans cell histiocytosis[26]
  • Clonal proliferation of Langerhans cells
  • Any age
  • More common in children between 5-10 years old
  • Boys>girls
- - + + - Nl Nl + - Nl
  • Osteolytic lesions on imaging
  • Lung nodules on imaging
Tissue biopsy
Category Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings

Diagnosis

Treatment

If the splenomegaly underlies hypersplenism, a splenectomy is indicated and it will correct the problem. After splenectomy, patients have an increased risk for infectious diseases. After splenectomy, patients should be vaccinated against Haemophilus influenzae and Streptococcus pneumoniae. They should receive annual influenza vaccination. Long-term prophylactic antibiotic should be given.

References

  1. Wu Z, Zhou J, Pankaj P, Peng B (December 2012). "Laparoscopic and open splenectomy for splenomegaly secondary to liver cirrhosis: an evaluation of immunity". Surg Endosc. 26 (12): 3557–64. doi:10.1007/s00464-012-2366-5. PMID 22710653.
  2. Gedela M, Khan M, Jonsson O (September 2015). "Heart Failure". S D Med. 68 (9): 403–5, 407–9. PMID 26489162.
  3. Ferri PM, Ferreira AR, Fagundes ED, Liu SM, Roquete ML, Penna FJ (2012). "Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center". Arq Gastroenterol. 49 (1): 69–76. PMID 22481689.
  4. Ishibashi N, Maebayashi T, Aizawa T, Sakaguchi M, Abe O, Saito T, Tanaka Y (May 2015). "Myelosuppression toxicity of palliative splenic irradiation in myelofibrosis and malignant lymphoma". Hematology. 20 (4): 203–7. doi:10.1179/1607845414Y.0000000192. PMID 25131182.
  5. Quest GR, Johnston JB (December 2015). "Clinical features and diagnosis of hairy cell leukemia". Best Pract Res Clin Haematol. 28 (4): 180–92. doi:10.1016/j.beha.2015.10.017. PMID 26614896.
  6. Chou YS, Gau JP, Yu YB, Pai JT, Hsiao LT, Liu JH, Hong YC, Liu CY, Yang CF, Chen PM, Chiou TJ, Tzeng CH (March 2013). "Leukocytosis in polycythemia vera and splenomegaly in essential thrombocythemia are independent risk factors for hemorrhage". Eur. J. Haematol. 90 (3): 228–36. doi:10.1111/ejh.12064. PMID 23281576.
  7. Grieco A, Manna R, Mancini R, Gambassi G (April 1988). "Massive hepatomegaly following splenectomy for myeloid metaplasia". Am. J. Med. 84 (4): 797. PMID 3400674.
  8. Tefferi A, Barbui T (February 2015). "Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management". Am. J. Hematol. 90 (2): 162–73. doi:10.1002/ajh.23895. PMID 25611051.
  9. Cervantes F, Martinez-Trillos A (May 2013). "Myelofibrosis: an update on current pharmacotherapy and future directions". Expert Opin Pharmacother. 14 (7): 873–84. doi:10.1517/14656566.2013.783019. PMID 23514013.
  10. Adachi K, Ui M, Nojima H, Takada Y, Enatsu K (August 2011). "Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy". Am. J. Surg. 202 (2): e17–9. doi:10.1016/j.amjsurg.2010.10.002. PMID 21810495.
  11. Huprikar NA, Kurtz MT, Mount CA (October 2013). "Massive splenomegaly and lymphopenia: a unique case of obstructive shock". BMJ Case Rep. 2013. doi:10.1136/bcr-2013-201643. PMC 3822246. PMID 24172780.
  12. Kumar KJ, Kumar HC, Manjunath VG, Anitha C, Mamatha S (January 2014). "Hepatitis A in children- clinical course, complications and laboratory profile". Indian J Pediatr. 81 (1): 15–9. doi:10.1007/s12098-013-1114-8. PMID 23904062.
  13. Gao LW, Xie ZD, Liu YY, Wang Y, Shen KL (February 2011). "Epidemiologic and clinical characteristics of infectious mononucleosis associated with Epstein-Barr virus infection in children in Beijing, China". World J Pediatr. 7 (1): 45–9. doi:10.1007/s12519-011-0244-1. PMID 21191775.
  14. Smiechura M, Strużycka M, Konopka W (2014). "Congenital and acquired cytomegalovirus infection and hearing evaluation in children". Otolaryngol Pol. 68 (6): 303–7. doi:10.1016/j.otpol.2014.04.003. PMID 25308607.
  15. Rosche KL, Aljasham AT, Kipfer JN, Piatkowski BT, Konjufca V (2015). "Infection with Salmonella enterica Serovar Typhimurium Leads to Increased Proportions of F4/80+ Red Pulp Macrophages and Decreased Proportions of B and T Lymphocytes in the Spleen". PLoS ONE. 10 (6): e0130092. doi:10.1371/journal.pone.0130092. PMC 4466801. PMID 26068006.
  16. Roset MS, Ibañez AE, de Souza Filho JA, Spera JM, Minatel L, Oliveira SC, Giambartolomei GH, Cassataro J, Briones G (2014). "Brucella cyclic β-1,2-glucan plays a critical role in the induction of splenomegaly in mice". PLoS ONE. 9 (7): e101279. doi:10.1371/journal.pone.0101279. PMC 4077732. PMID 24983999.
  17. Chandni R, Chandni R, Chandni R (2016). "Extra pulmonary tuberculosis presenting as fever with massive splenomegaly and pancytopenia". IDCases. 4: 20–2. doi:10.1016/j.idcr.2016.02.005. PMC 4802818. PMID 27051579.
  18. Sumbele IU, Bopda OS, Kimbi HK, Ning TR, Nkuo-Akenji T (November 2015). "Influence of Plasmodium gametocyte carriage on the prevalence of fever, splenomegaly and cardiovascular parameters in children less than 15 years in the Mount Cameroon area: cross sectional study". BMC Infect. Dis. 15: 547. doi:10.1186/s12879-015-1290-4. PMC 4661959. PMID 26612502.
  19. Sayyahfar S, Ansari S, Mohebali M, Behnam B (April 2014). "Visceral leishmaniasis without fever in an 11-month-old infant: a rare clinical feature of Kala-azar". Korean J. Parasitol. 52 (2): 189–91. doi:10.3347/kjp.2014.52.2.189. PMC 4028457. PMID 24850963.
  20. Modaresi Esfeh J, Culver D, Plesec T, John B (March 2015). "Clinical presentation and protocol for management of hepatic sarcoidosis". Expert Rev Gastroenterol Hepatol. 9 (3): 349–58. doi:10.1586/17474124.2015.958468. PMID 25473783.
  21. Chiong FJ, Loewenthal M, Boyle M, Attia J (December 2015). "Serum sickness-like reaction after influenza vaccination". BMJ Case Rep. 2015. doi:10.1136/bcr-2015-211917. PMC 4691861. PMID 26677148.
  22. Yu C, Gershwin ME, Chang C (2014). "Diagnostic criteria for systemic lupus erythematosus: a critical review". J. Autoimmun. 48-49: 10–3. doi:10.1016/j.jaut.2014.01.004. PMID 24461385.
  23. Huang T, Liu M, Xu G (January 2014). "Felty's syndrome with hyperthyroidism: a case report and literature review". Int J Rheum Dis. 17 (1): 122–4. doi:10.1111/1756-185X.12117. PMID 24472276.
  24. Ayto R, Hughes DA (2013). "Gaucher disease and myeloma". Crit Rev Oncog. 18 (3): 247–68. PMID 23510067.
  25. Vanier MT (2013). "Niemann-Pick diseases". Handb Clin Neurol. 113: 1717–21. doi:10.1016/B978-0-444-59565-2.00041-1. PMID 23622394.
  26. DiCaprio MR, Roberts TT (October 2014). "Diagnosis and management of langerhans cell histiocytosis". J Am Acad Orthop Surg. 22 (10): 643–52. doi:10.5435/JAAOS-22-10-643. PMID 25281259.