Splenomegaly

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Splenomegaly Classification and external resources
ICD-10	Q89.0, R16.1
ICD-9	759.0, 789.2
DiseasesDB	12375
MedlinePlus	003276
eMedicine	ped/2139  med/2156
MeSH	D013163

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Splenomegaly is an enlargement of the spleen, which usually lies in the left upper quadrant (LUQ) of the human abdomen. It is one of the four cardinal signs of hypersplenism, the other three being cytopenia(s), normal or hyperplastic bone marrow, and a response to splenectomy. Splenomegaly is usually associated with increased workload (such as in hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas.

Complete Differential Diagnosis Splenomegaly

• AIDS
• Angioimmunoblastic lymphoanedopathy
• Angiosarcoma
• Autoimmune hemolytic anemia
• Bacterial septicemia
• Bone marrow damage
• Bone marrow infiltration
• Brucellosis
• Castleman's syndrome
• Cavernous transformation of the portal vein
• Cellular infiltration
• Chronic myoletic leukemia
• Collagen vascular diseases
• Congestive heart failure
• Constrictive pericarditis
• Coronavirus
• Corynebacterium diphtheriae
• Cytomegalovirus
• Early sickle cell anemia
• Endocarditis
• Ehrlichiosis
• Eosinophillic granuloma
• Epstein-Barr Virus Infection
• Felty's syndrome
• Fever unknown origin
• Fibromas
• Fungal infections
• Gaucher's disease
• Hemangiomas
• Hamartomas
• Hemoglobinopathy
• Hemolytic anemia
• Hepatic echinococcosis
• Hepatic schistomasis
• Hepatic vein obstruction
• Hepatitis
• Hereditary spherocytosis
• Histiocytosis
• Histoplasmosis
• HIV
• Hodgkin's lymphoma
• Hurler's syndrome
• Hyperlipidemias
• Idiopathic splenomegaly
• Immune hemolytic anemias
• Infective Endocarditis
• Infectious mononucleosis
• Interleukin-2
• Iron deficiency anemia
• Leishmaniasis
• Letterer-Siwe disease
• Leukemia
• Lymphangiomas
• Lymphoid leukemia
• Lympho-reticulosarcoma
• Malaria
• Malignancy
• Melanoma
• Myelofibrosis
• Myeloid leukemia
• Myeloid metaplasia
• Monocytic leukemia
• Mononucleosis
• Myobacterium avium complex
• Myoproliferative syndrome(s)
• Niemann-Pick disease
• Non-Hodgkin's lymphoma
• Nutritional anemias
• Osteomyelosclerosis
• Paroxysmal nocturnal hemoglobinuria
• Polycythemia vera
• Portal hypertension
• Q fever
• Radiation
• Rheumatoid arthritis
• RMSF
• Sarcoidosis
• Schistosomiasis
• Serum sickness
• Sickle cell disease
• Splenic abscess
• Splenic artery anuerysm
• Splenic cysts
• Splenic hamartoma
• Splenic hemangioma
• Splenic vein obstruction/thrombosis
• Stillness disease
• Subacute bacterial endocarditis
• Syphillis
• Systemic lupus erythematosus
• Tangier disease
• Thalassemia major
• Thyrotoxicosis
• Toxoplasmosis
• Trauma
• Trypanosomiasis
• Tuberculosis
• Tumors
• Typhoid fever
• Vein obstruction
• Viral hepatitis
• Weil's disease
• Less common causes ^[1]

  • Amyloidosis
  • Babesiosis
  • Gaucher's disease
  • Histoplasmosis
  • Kala-azar
  • Rickets
  • Schistosomiasis
  • Syphilis
  • Toxoplasmosis
  • Typhoid fever

Symptoms and signs

Symptoms may include abdominal pain, early satiety due to splenic encroachment, or the symptoms of anemia due to accompanying cytopenia.

Signs of splenomegaly may include a palpable left upper quadrant abdominal mass or splenic rub. It can be detected on physical examination by using Castell's sign or Traube's space, but an ultrasound can be used to confirm diagnosis.^[2]

Causes

Splenomegaly grouped on the basis of the pathogenic mechanism

Increased function	Abnormal blood flow	Infitration
Removal of defective RBCs Spherocytosis Thalassemia Hemoglobinopathies Nutritional anemias early sickle cell anemia Immune hyperplasia Response to infection (viral,bacterial,fungal,parasitic) Mononucleosis, AIDS, viral hepatitis subacute bacterial endocarditis, bacterial septicemia splenic abscess, typhoid fever brucellosis, leptospirosis, tuberculosis histoplasmosis malaria, leishmaniasis, trypanosomiasis ehrlichiosis Disordered immunoregulation Rheumatoid arthritis SLE Serum sickness Autoimmune hemolytic anemia Immune thrombocytopenia sarcoidosis drug reactions Extramedullary hematopoiesis Myelofibrosis Marrow infiltration by tumors, leukemias marrow damage by radiation, toxins	Organ Failure Cirrhosis congestive heart failure Vascular hepatic vein obstruction portal vein obstruction Budd-Chiari syndrome splenic vein obstruction Infections hepatic schistosomiasis hepatic echinococcosis	Metabolic diseases Gauchers disease Niemann-Pick disease Hurler syndrome and other Mucopolysaccharidoses Amyloidosis Tangier disease Benign and malignant infiltrations Leukemias(acute,chronic,lymphoid and myeloid) lymphomas(Hodgkins and non-hodgkins) myeloproliferative disorders metastatic tumors(commonly melanoma) histiocytosis X Hemangioma,lymphangioma splenic cysts hamartomas eosinophilic granuloma

The causes of massive splenomegaly (>1000gms) are much fewer and include:

Thalassemia
Kala-Azar (Leishmaniasis)
Portal hypertension of Bilharziasis
Chronic myelogenous leukemia
lymphomas
hairy cell leukemia
myelofibrosis
polycythemia vera
Gauchers disease
chronic lymphocytic leukemia
sarcoidosis
autoimmune hemolytic anemia
Malaria

Treatment

If the splenomegaly underlies hypersplenism, a splenectomy is indicated and will correct the problem. After splenectomy, however, 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 vaccinations. Long-term prophylactic antibiotics should be given.

See also

• sign (medicine)
• Hepatosplenomegaly

References

1. ↑ Kahan, Scott, Smith, Ellen G. In a page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:157
2. ↑ Grover SA, Barkun AN, Sackett DL (1993). "The rational clinical examination. Does this patient have splenomegaly?". JAMA 270 (18): 2218-21. PMID 8411607. Ovid full text

External links

• PatientPlus Splenomegaly and hypersplenism
• 11-141b. at Merck Manual of Diagnosis and Therapy Professional Edition (Hypersplenism)

v • d • e Phakomatoses and other congenital malformations not elsewhere classified (Q85-Q89, 759)
Phakomatoses	Abdallat Davis Farrage syndrome - Ataxia telangiectasia - Incontinentia pigmenti - Neurofibromatosis (type I, type II) - Peutz-Jeghers syndrome - Sturge-Weber syndrome - Tuberous sclerosis - Von Hippel-Lindau disease
Due to known exogenous causes	Fetal alcohol syndrome - Phocomelia (via Thalidomide)
Affecting multiple systems	facial (Mobius syndrome, Goldenhar syndrome, Cyclopia, Apert syndrome) short stature (Aarskog-Scott syndrome, Cockayne syndrome, Cornelia de Lange Syndrome, Dubowitz syndrome, Noonan syndrome, Robinow syndrome, Silver-Russell dwarfism, Seckel syndrome, Smith-Lemli-Opitz syndrome) limbs (Adducted thumb syndrome, Holt-Oram syndrome, Klippel-Trenaunay-Weber syndrome, Nail-patella syndrome, Rubinstein-Taybi syndrome, Sirenomelia, VACTERL association) overgrowth (Beckwith-Wiedemann syndrome, Sotos syndrome, Weaver syndrome) combined/other Ablepharon macrostomia syndrome - Alport syndrome - Bardet-Biedl syndrome - Branchio-oto-renal syndrome - Donohue syndrome - Fraser syndrome - Keutel syndrome - Marfan syndrome - Timothy syndrome - Urban-Rogers-Meyer syndrome - Vici syndrome - Yunis-Varon syndrome - Zellweger syndrome - Zimmerman-Laband syndrome - Zori Stalker Williams syndrome
Other	spleen: Asplenia - Splenomegaly endocrine glands: Persistent thyroglossal duct - Thyroglossal cyst Conjoined twins - Cowden syndrome - Hamartoma - Impossible syndrome - Situs inversus

v • d • e Symptoms and signs (R00-R69, 780-789)
Circulatory and respiratory systems	Tachycardia - Bradycardia - Palpitation - Heart murmur - Nosebleed - Hemoptysis - Cough - abnormalities of breathing (Dyspnea, Orthopnoea, Stridor, Wheeze, Cheyne-Stokes respiration, Hyperventilation, Mouth breathing, Hiccup, Bradypnea, Hypoventilation) - Chest pain - Asphyxia - Pleurisy - Respiratory arrest - Sputum - Bruit
Digestive system and abdomen	Abdominal pain - Acute abdomen - Nausea - Vomiting - Heartburn - Dysphagia - Flatulence - Burping - Fecal incontinence - Encopresis - Hepatomegaly - Splenomegaly - Hepatosplenomegaly - Jaundice - Ascites - Fecal occult blood - Halitosis
Skin and subcutaneous tissue	disturbances of skin sensation (Hypoesthesia, Paresthesia, Hyperesthesia) - Rash - Cyanosis - Pallor - Flushing - Petechia - Desquamation - Induration
Nervous and musculoskeletal systems	abnormal involuntary movements (Tremor, Spasm, Fasciculation, Athetosis) - Gait abnormality - lack of coordination (Ataxia, Dysmetria, Dysdiadochokinesia, Hypotonia) - Tetany - Meningism - Hyperreflexia
Urinary system	Dysuria - Vesical tenesmus - Urinary incontinence - Urinary retention - Oliguria - Polyuria - Nocturia
Cognition, perception, emotional state and behaviour	Anxiety - Somnolence - Coma - Amnesia (Anterograde amnesia, Retrograde amnesia) - Dizziness/Vertigo - smell and taste (Anosmia, Ageusia, Parosmia, Parageusia)
Speech and voice	speech disturbances (Dysphasia, Aphasia, Dysarthria) - symbolic dysfunctions (Dyslexia, Alexia, Agnosia, Apraxia, Acalculia, Agraphia) - voice disturbances (Dysphonia, Aphonia)
General symptoms and signs	Fever (Hyperpyrexia) - Headache - Chronic pain - Malaise - Fatigue - Fainting (Vasovagal syncope) - Febrile seizure - Shock (Cardiogenic shock) - Lymphadenopathy - Edema (Peripheral edema, Anasarca) - Hyperhidrosis (Sleep hyperhidrosis) - Delayed milestone - Failure to thrive - food and fluid intake (Anorexia, Polydipsia, Polyphagia) - Cachexia - Xerostomia - Clubbing

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .