Imaging Pearls ❯ Spleen ❯ Splenomegaly
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- Splenomegaly: Causes
- Viral infections, such as mononucleosis
- Bacterial infections, such as syphilis or an infection of your heart's inner lining (endocarditis)
- Parasitic infections, such as malaria
- Cirrhosis and other diseases affecting the liver
- Various types of hemolytic anemia — a condition characterized by early destruction of red blood cells
- Blood cancers, such as leukemia and myeloproliferative neoplasms, and lymphomas, such as Hodgkin's disease
- Metabolic disorders, such as Gaucher disease and Niemann-Pick disease
- Pressure on the veins in the spleen or liver or a blood clot in these veins
- Autoimmune conditions, such as lupus or sarcoidosis - “Splenomegaly is a common finding in multiple diseases; however, massive enlargement of the spleen is seen in few conditions. Most authors define massive splenomegaly when the spleen reaches the iliac crest, crosses the midline or weights more than 1500 g. The most common aetiologies of massive splenomegaly include haematological disorders (chronic myeloid leukamia, agnogenic myeloid metaplasia, polycythaemia vera, essential thrombocythaemia, indolent lymphomas, hairy cell leukaemia, β-thalassaemia major), infectious diseases (visceral leishmaniasis, malaria) and infiltrative conditions (Gaucher disease).”
Massive splenomegaly.
Paz-Y-Mar HL, Gonzalez-Estrada A, Alraies MC.
BMJ Case Rep. 2013 Jul 29;2013:bcr2013200515.
- “The radiographic features of b-thalassemia are due in large part to marrow hyperplasia. Markedly expanded marrow space lead to various skeletal manifestations including spine, skull, facial bones, and ribs. Extramedullary hematopoiesis (ExmH), hemosiderosis, and cholelithiasis are among the non-skeletal manifestations of thalassemia. The skeletal X-ray findings show characteristics of chronic overactivity of the marrow.”
Imaging features of thalassemia
M. Tunacõ et al.
Eur. Radiol. 9, 1804±1809 (1999) - “The thalassemias are hereditary anemias that occur because of mutations that affect the synthesis of hemoglobin and is typically seen in Mediterranean countries. Thalassemias can be classified according to the chain involved. In b-thalassemia there is deficient synthesis of b-globin, whereas in a-thalassemia there is deficient synthesis of a-globin. Reduced synthesis of one of the two globin polypeptides leads to deficient hemoglobin accumulation, resulting in hypochromic and microcytic red cells.”
Imaging features of thalassemia
M. Tunacõ et al.
Eur. Radiol. 9, 1804±1809 (1999) - “Extramedullary hematopoiesis represents the body's attempt to maintain erythrogenesis when there is an important alteration in blood cell population. In thalassemia, posterior mediastinal paravertebral masses or presacral masses represent sites of ExmH resulting from extraosseous extension of medullary tissue. Also ExmH can arise from pluripotential stem cells distributed throughout the body, and involvement of the abdominal viscera, including liver, spleen, kidneys, adrenal glands, and breast, may occur.”
Imaging features of thalassemia
M. Tunacõ et al.
Eur. Radiol. 9, 1801-1809 (1999) - “The severity of osseous changes is dependent on the thalassaemia subtype, duration of disease as well as transfusion–chelation treatment. Changes overall are most pronounced in undertreated TDT. Marrow hyperplasia secondary to chronic anaemia results in medullary expansion, cortical bone thinning, cancellous bone resorption as well as osteopaenia, which may result in pathological fractures.Osteopaenia remains a prominent cause of morbidity, even in optimally treated patients.”
Imaging features of thalassaemia
Maria Gosein et al.
Br J Radiol. 2019 Apr; 92(1096): 20180658. - “Extramedullary haematopoiesis (EMH) EMH may be classified as paraosseous or extraosseous and is more common in NTDT than in regularly transfused TDT.Paraosseous EMH typically occurs in the thoracic region, followed by the lumbar region. This appears as lobulated posterior mediastinum soft-tissue masses involving the posterior rib segments, or less often the anterior ends of ribs or presacral region.”
Imaging features of thalassaemia
Maria Gosein et al.
Br J Radiol. 2019 Apr; 92(1096): 20180658. - "Extraosseous EMH is thought to arise from pluripotential stem cells, most commonly affecting the liver and spleen, resulting in hepatosplenomegaly or pseudotumours when mass-like.EMH can also occur in lymph nodes or less commonly in the kidneys, CNS, adrenals, mesentery, middle ear, paratracheal region, thymus, heart, breasts, prostate, and broad ligaments.”
Imaging features of thalassaemia
Maria Gosein et al.
Br J Radiol. 2019 Apr; 92(1096): 20180658. - "Massive splenomegaly carries risks of splenic rupture or hypersplenism. Splenomegaly due to undertransfusion may be reversible. Although there is potential reduction in transfusional iron loading after splenectomy, this must be weighed against long-term asplenia consequences. Accessory spleens occur in greater frequency in haematologic disorders. They are commonly located at the splenic hilum, but can occur throughout the abdomen, occasionally mimicking tumours. On cross-sectional imaging, they appear similar to normal splenic tissue on all phases. Tc-99m heat-damaged RBCs or sulphur colloid scans can aid in diagnosis.”
Imaging features of thalassaemia
Maria Gosein et al.
Br J Radiol. 2019 Apr; 92(1096): 20180658. - Extramedullary Haematopoiesis (EMH): Causes
-hereditary spherocytosis
-sickle cell anemia
-congenital dyserythropoietic anemia
-immune thrombocytopenic purpura
-chronic myeloid leukemia polycythemia vera
-myelodysplastic syndrome
-Paget disease
-osteopetrosis
-Gaucher disease
-treatment with myeloid growth factors - “EMH refers to the formation and growth of hematopoietic elements outside the bone marrow, secondary to the insufficient erythropoiesis like myelofibrosis with myeloid metaplasia and thalassemia, also associating with many other disorders including hereditary spherocytosis, sickle cell anemia, congenital dyserythropoietic anemia, immune thrombocytopenic purpura, chronic myeloid leukemia, polycythemia vera, myelodysplastic syndrome, Paget disease, osteopetrosis, and Gaucher disease, and treatment with myeloid growth factors.”
A large intrathoracic extramedullary hematopoiesis in alpha-thalassemia: A case report
Jianan Chen et al.
Medicine (Baltimore). 2019 Nov; 98(44): e17612. - “EMH is a reactive or compensatory mechanism of new extramarrow blood element formation because of chronically ineffective erythropoiesis. It is usually seen in paraspinal areas, the spleen, liver, and lymph nodes, although a spectrum of other anatomical sites has been reported, as described previously. The rarity of the disease and the nonspecific findings hinder initial consideration of the diagnosis. Physicians need to be aware of this possibility to initiate appropriate diagnostic and treatment interventions. Knowledge of the patients’ clinical history is absolutely essential.”
Extramedullary Hemopoiesis
EleniOrphanidou-Vlachou et al.
Seminars in Ultrasound, CT and MRI Volume 35, Issue 3, June 2014, Pages 255-262 - "Various chronic hematologic disorders that lead to ineffective hemopoiesis or inadequate bone marrow function (ie, chronic hemolytic anemias, thalassemia, sickle cell anemia, myelofibrosis of many causes, lymphoma, and leukemia) can potentially precipitate extramarrow new blood element creation. Extramarrow soft tissue that produces blood elements is called extramedullary hemopoietic tissue and the process extramedullary hemopoiesis (EMH).”
Extramedullary Hemopoiesis
EleniOrphanidou-Vlachou et al.
Seminars in Ultrasound, CT and MRI Volume 35, Issue 3, June 2014, Pages 255-262 - “Extramarrow soft tissue that produces blood elements is called extramedullary hemopoietic tissue and the process extramedullary hemopoiesis (EMH). Sites commonly involved by EMH include the liver, spleen, lymph nodes, and most commonly, paravertebral regions, although other sites can sometimes be involved. Physicians rarely consider EMH in their differential diagnosis even in cases where it is warranted (diseases of ineffective erythropoiesis). This is likely because of the rarity of the condition and because imaging findings are nonspecific.”
Extramedullary Hemopoiesis
EleniOrphanidou-Vlachou et al.
Seminars in Ultrasound, CT and MRI Volume 35, Issue 3, June 2014, Pages 255-262 - “On CT images, EMH paravertebral masses appear isodense to adjacent muscle, smooth and well marginated and usually do not erode adjacent bone from outside-in. Actively hemopoietic masses demonstrate mild homogeneous enhancement after contrast (high vascularity), whereas old burnt-out lesions have inhomogeneous contrast enhancement owing to iron deposition and fat infiltration.These paraspinal masses are characteristic as they involve multiple levels in a bilateral distribution and are nearly symmetric.”
Extramedullary Hemopoiesis
EleniOrphanidou-Vlachou et al.
Seminars in Ultrasound, CT and MRI Volume 35, Issue 3, June 2014, Pages 255-262 - “The commonest sites of EMH in the abdomen are the liver, spleen, and lymph nodes, all sites of in utero hemopoietic activity. Signs and symptoms include hepatosplenomegaly and abdominal discomfort, as well as shortness of breath in the case of massive enlargement. Pain can be the presenting symptom if splenic infarction occurs. Hepatosplenomegaly can be detected on cross-sectional imaging (CT, MRI, and ultrasound) and 99mTc methylene diphosphonate whole-body bone scan.”
Extramedullary Hemopoiesis
EleniOrphanidou-Vlachou et al.
Seminars in Ultrasound, CT and MRI Volume 35, Issue 3, June 2014, Pages 255-262
- “EMH occurs secondary to deficient bone marrow cells and arises from pluripotential stem cells distributed throughout the body. EMH can occur in congenital hemolytic disorders, with EMH presenting adjacent to hematopoietically active bones. When EMH occurs in patients with acquired marrow replacement disorders such as myeloproliferative diseases, the marrow space is nonfunctional and EMH can occur in organs such as the spleen or liver and lymph nodes. EMH in the spleen is usually diffusely infiltrative but can present as a focal masslike lesion with reported sizes ranging from 2.5 to 7.0 cm.”
Nonneoplastic, Benign, and Malignant Splenic Diseases: Cross-Sectional Imaging Findings and Rare Disease Entities Thipphavong S et al. AJR 2014; 203:315–322 - “Splenic metastases can occur with wide- spread disease, and parenchymal disease is caused by hematogenous dissemination. The most common primary cancers with splenic metastases include melanoma and cancers of the breast, lung, ovary, stomach, and prostate.
On ultrasound and CT, splenic metastases are typically hypoechoic and hypodense, respectively, but can vary depending on the primary tumor. On MRI, metastases usually have low signal intensity on T1-weighted imaging and high signal intensity on T2-weight- ed imaging. Metastases show varying degrees of enhancement. Peritoneal disease can deposit on the capsular surface of the spleen, which is most commonly seen with gynecologic malignancies.”
Nonneoplastic, Benign, and Malignant Splenic Diseases: Cross-Sectional Imaging Findings and Rare Disease Entities Thipphavong S et al. AJR 2014; 203:315–322 - “The most common primary benign vascular neoplasm of the spleen is hemangioma. Other benign vascular neoplasms of the spleen include hamartoma, lymphangioma, EMH, and SANT. Primary splenic angiosarcoma is the most common malignant nonhematolymphoid malignancy of the spleen. Lymphoma, myeloma, and metastases are the other malignant entities involving the spleen.”
Nonneoplastic, Benign, and Malignant Splenic Diseases: Cross-Sectional Imaging Findings and Rare Disease Entities Thipphavong S et al. AJR 2014; 203:315–322
- “The purpose of this study was to evaluate the impact of cirrhosis etiology on spleen size as measured by sonography and computed tomography (CT).The spleen images of 139 patients with cirrhosis secondary to alcohol abuse, hepatitis C, or nonalcoholic steatohepatitis were reviewed retrospectively. The maximum diagonal spleen length on a single sonogram and maximum spleen diameter on axial, coronal, or sagittal CT, whichever was largest, was compared among the etiologic groups.”
Spleen size in cirrhosis of different etiologies
J Ultrasound Med. 2015 Feb;34(2):233-8
Kashani A et al. - “In 87 patients who underwent CT, the mean spleen size on CT in the alcohol group (14.0 ± 2.7 cm) was smaller than in the hepatitis C (15.9 ± 3.4 cm) and nonalcoholic steatohepatitis (15.5 ± 3.6 cm) groups, but the difference was not statistically significant.”
Spleen size in cirrhosis of different etiologies
J Ultrasound Med. 2015 Feb;34(2):233-8
Kashani A et al. - “Spleen size in patients with cirrhosis varies by the etiology of the disease. Therefore, to apply spleen size as a diagnostic or prognostic criterion in this context, it is important to recognize that cutoff values derived from spleen size in one etiologic group may not produce the same results when extrapolated to another etiologic group.”
Spleen size in cirrhosis of different etiologies
J Ultrasound Med. 2015 Feb;34(2):233-8
Kashani A et al.
- “ Intrapancreatic accessory spleen (IPAS) should be considered when a hypervascular mass is seen in the tail of the pancreas on CT. Typical location, similar attenuation of the lesion to the spleen on noncontrast, and postcontrast CT at different phases are helpful to make diagnosis of IPAS.”
Intrapancreatic accessory spleen: CT appearance and differential diagnosis
Kawamoto S, Johnson PT, Hull H, Cameron JL, Hruban RH, Fishman EK
Abdom Imaging (2012)37:812-827 - “ In autopsy studies, the second most common site of accessory spleen is the tail of the pancreas.”
Intrapancreatic accessory spleen: CT appearance and differential diagnosis
Kawamoto S, Johnson PT, Hull H, Cameron JL, Hruban RH, Fishman EK
Abdom Imaging (2012)37:812-827
- Gaucher's disease is a rare, inherited disorder that causes too much of a substance called glucocerebroside to build up in your spleen, liver, lungs, bones and sometimes in your brain. The buildup prevents these organs from working properly. There are three types:
Type 1, the most common form, causes liver and spleen enlargement, bone pain and broken bones, and, sometimes, lung and kidney problems. It does not involve the brain. It can occur at any age.
Type 2, which causes severe brain damage, appears in infants. Most children who have it die by age 2. In type 3, there may be liver and spleen enlargement, and signs of brain involvement appear gradually.
Medline Plus from NIH - Gauchers Disease: CT Findings
- Splenomegaly
- Focal splenic lesions
- Diffuse infiltration
- Concurrent splenic and hepatic involvement may occur
- Splenomegaly: Differential Dx
- Portal hypertension
- Infection (i.e. abscess)
- Neoplastic disease (i.e lymphoma)
- Blood disorders (i.e. polycythemia vera)
- Storage diseases (i.e. Gauchers disease) - Splenomegaly: Differential Dx
Tumor
- leukemia
- lymphoma
Infections
- mononucleosis, CMV
- Granulomatous disease (TB, Histo)
Congestion
- portal hypertension