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 |
|
NA | Bone marrow examination + clinical manifestation |
| |||
Polycythemia Vera[6] |
|
Mean age >60 years old |
|
– | + | Nl | + | – | Nl | Nl | – | – |
|
Nl to ↑ | ↑ | Nl to ↑ | High ferritin | Nl | Nl | Nl | Nl |
|
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 | ↑ | ↑ |
|
Bone marrow examination |
| ||||||
Essential thrombocythemia[8] |
|
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 |
|
+ | + | ↓ | + | – | Nl | Nl | – | – |
|
↓ | ↓ | ↓ | Low iron, low ferritin | ↑ | ↑ | ↑ |
|
|
Biopsy |
| ||||
Metastatic solid tumors[11] |
|
Any age, more in adults |
|
+ | + | ↓ | + | + | ↓ | Nl | ± | ± | ↓ | ↓ | ↓ | Low iron, high ferritin | ↑ | ↑ | ↑ |
|
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] |
|
|
+ | + | ↓ | + | + | Nl | Nl | + | + |
|
↓ | ↓ | ↓ | Nl | ↑ | ↑ | ↑ | NA | Clinical manifestation + lab tests |
| |||
Infectious mononucleosis[13] |
|
|
+ | + | Nl | + | – | Nl | Nl | + | – |
|
↑ | Nl | ↑ | Nl | ↑ | Nl | Nl | Nl | NA | NA | Clinical manifestation + heterophile antibody test | ||||
Cytomegalovirus[14] |
|
|
+ | + | Nl | + | – | Nl | Nl | + | – |
|
↑ | Nl | ↑ | Nl | ↑ | Nl | Nl | Nl | NA | NA | ELISA | ||||
Bacterial | Salmonella[15] |
|
|
+ | – | Nl | + | – | ↓ | Nl | – | – |
|
↑ | Nl | ↑ | Nl | Nl | Nl | ↑ | NA | NA | Stool test |
| |||
Brucella[16] |
|
|
|
+ | + | Nl | + | – | ↓ | Nl | + | – |
|
↑ | Nl | ↑ | Nl | ↑ | Nl | Nl | NA | NA | Blood culture |
| |||
Tuberculosis[17] |
|
|
|
+ | + | ↓ | + | – | ↓ | Nl | + | – |
|
↑ | Nl | ↑ | Nl | ↑ | ↑ | ↑ |
|
|
Identifying M. tuberculosis in a clinical sample |
| |||
Parasitic | Malaria[18] |
|
|
|
+ | + | Nl | + | + | ↓ | Nl | + | – | ↑ | ↓ | ↑ | Nl | ↑ | Nl | ↑ | NA | ||||||
Kala-azar[19] |
|
|
+ | + | ↓ | + | + | ↓ | Nl | + | + |
|
↓ | ↓ | ↓ | Nl | ↑ | ↑ | ↑ |
|
NA | Culture |
| ||||
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] |
|
|
|
+ | + | ↓ | + | + | Nl | Nl | – | – | ↑ | ↓ | ↑ | Nl | Nl | Nl | Nl | Nl | NA |
|
Diagnosis of exclusion |
| ||
Serum sickness[21] |
|
|
|
+ | + | Nl | + | – | ↓ | Nl | – | – | ↓ or ↑
Eosinophilia |
↓ | ↑ | Nl | Nl | Nl | ↑ |
|
NA | Clinical manifestation + recent medication consumption | |||||
Systemic lupus erythematosus[22] |
|
|
|
+ | + | ↓ | + | + | Nl to ↓ | Nl | + | + | ↓ | ↓ | ↓ | ↑ | ↑ | ↑ |
|
Clinical findings + laboratory studies |
| ||||||
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] |
|
|
|
- | + | ↓ | + | + | Nl to ↓ | Nl | + | + |
|
↓ | ↓ | ↓ | Nl | Nl | ↑ | ↑ |
|
NA | Enzyme testing |
| |||
Niemann-Pick disease[25] |
|
|
|
- | + | ↑ | + | + | Nl to ↓ | Nl | + | + |
|
↓ | ↓ | ↓ | Nl | ↑ | ↑ | ↑ |
|
NA | Genetic testing |
| |||
Langerhans cell histiocytosis[26] |
|
|
- | - | + | ↓ | + | - | Nl | Nl | + | - |
|
↓ | ↓ | ↓ | Nl | ↑ | ↑ | ↑ |
|
|
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
- ↑ 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.
- ↑ Gedela M, Khan M, Jonsson O (September 2015). "Heart Failure". S D Med. 68 (9): 403–5, 407–9. PMID 26489162.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Ayto R, Hughes DA (2013). "Gaucher disease and myeloma". Crit Rev Oncog. 18 (3): 247–68. PMID 23510067.
- ↑ Vanier MT (2013). "Niemann-Pick diseases". Handb Clin Neurol. 113: 1717–21. doi:10.1016/B978-0-444-59565-2.00041-1. PMID 23622394.
- ↑ 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.