eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Asplenia: Differential Diagnoses & Workup

Author: Joseph C Turbyville, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center
Coauthor(s): Cecilia P Mikita, MD, MPH, Associate Program Director, Allergy-Immunology Fellowship, Chief, Clinical Services, Allergy-Immunology Clinic, Walter Reed Army Medical Center; Mudra Kumar, MD, MBBS, MRCP, Associate Professor, Department of Pediatrics, University of South Florida College of Medicine
Contributor Information and Disclosures

Updated: Aug 13, 2008

Differential Diagnoses

Other Problems to Be Considered

Immune deficiency
Aberrant (wandering) spleen

Workup

Laboratory Studies

  • Often the first clues to functional asplenia in an asymptomatic patient are abnormalities in the peripheral blood smear.
    • The initial evaluation should begin with a review to identify HJ bodies (see Media file 1).
    • The nuclear remnants are small, round, densely stained inclusions in RBCs. They can be seen as a normal variant in the newborn period and are occasionally seen in leukemia, steatorrhea, and a variety of anemias (megaloblastic anemia, dyserythropoietic anemia, thalassemia).
    • The presence of HJ bodies in the peripheral smear of an individual older than 7 days should suggest splenic dysfunction.
    • Other red cell abnormalities include an increased presence of target cells, Heinz bodies, Pappenheimer bodies (small basophilic inclusions that contain nonheme iron), reticulocytes, and spherocytes.
    • In addition, thrombocytosis and leukocytosis are observed because the spleen functions as a reservoir for these blood cells.
  • Another helpful means of screening the splenic function is by determining the pit (autophagic vacuoles) count (ie, counting the number of pocked erythrocytes).
    • Normally, less than 2% of red cells have these pocks or pits. A pocked erythrocyte count of more than 3.5% suggests functional hyposplenia, and a count of more than 12% is indicative of asplenia.
    • This technique involves the use of special equipment (Nomarski optics) that is not always readily accessible.
  • An assessment of the argyrophilic granules in the peripheral smear may be a helpful and easy method to detect splenic dysfunction, but the procedure needs further evaluation.
  • HJ body quantitation by flow cytometry has also been described as a way to assay for splenic function but is not currently available, except in specialized laboratories.

Imaging Studies

  • Various imaging modalities may be helpful in defining splenic anomalies.
  • Certain associated anomalies, especially cardiac and visceral changes, often lead to further evaluation of the spleen.
  • Abdominal ultrasonography can be performed to document the presence of the spleen and its size, and newer ultrasonography techniques with color Doppler ultrasonography have shown promise in assessing splenic function. A small spleen with absent parenchymal vascularization on color Doppler ultrasonography has been associated with functional asplenia, but this should be confirmed with further imaging before declaring a patient functionally asplenic.
  • The absence of the spleen is best confirmed with a technetium-99m radionuclide scan. This agent is taken up by the reticuloendothelial cells and enables better assessment of splenic function. Similarly, as a result of functional asplenia, patients with sickle cell disease who still have normal-sized and even enlarged spleens demonstrate absent or decreased splenic uptake of technetium-99m sulfur colloid.
  • Absence of the intrahepatic segment of the inferior vena cava should trigger careful evaluation of abdominal masses, which could represent splenules. These masses can be confused with multiple metastatic lesions in patients, especially adults, in whom asplenia or polysplenia is undiagnosed.
  • MRI or CT scanning of the abdomen may also reveal absence or hypoplasia of the spleen. These studies have no place in the routine workup of isolated asplenia or hyposplenia but may be useful if they are obtained for other accompanying indications, such as visceral heterotaxy. Newer MRI techniques have expanded the role of MRI in the detection and characterization of splenic diseases.5
  • Computerized models are available to determine the splenic volume, but this approach is mainly used to judge the increased size (eg, tumors, infiltrations) of organs. With more experience, computerized models may provide important information about conditions that decrease the size and volume of the spleen.
  • If the spleen is not visualized with radiographic imaging and if no hematologic data support the diagnosis of asplenia, the extremely rare condition of wandering spleen may be considered. In this condition, a functional spleen is present; however, because of its long pedicle, it may be in an abnormal location, such as the pelvis.

Histologic Findings

  • A hypoplastic spleen may exhibit a hyperemic red pulp with underdevelopment of the white pulp and a paucity of lymphoid follicles.
  • When hyposplenia is secondary to an underlying hemoglobinopathy such as sickle cell disease, specific histologic features may be observed because of infarction (see History).

More on Asplenia

Overview: Asplenia
Differential Diagnoses & Workup: Asplenia
Treatment & Medication: Asplenia
Follow-up: Asplenia
Multimedia: Asplenia
References
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References

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Further Reading

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Keywords

asplenia, hypoplasia, splenic hypoplasia, absent spleen, nonfunctional spleen, autosplenectomy, hyposplenia, splen, Ivemark syndrome, asplenia syndrome, functional asplenia, congenital asplenia, bacterial sepsis, polysplenia, Klebsiella species, Escherichia coli, Streptococcus pneumoniae, Haemophilus influenzae type b, Neisseria meningitidis, malaria, babesiosis, endocardial cushion defects, pulmonary atresia, pulmonary stenosis, transposition of the great vessels, total anomalous pulmonary venous return

double-outlet right ventricle, atrioventricular canal defects, splenosis, respiratory distress, Pearson syndrome, pancreatic insufficiency, sideroblastic anemia, Stormorken syndrome, thrombocytopenia, miosis, Smith-Fineman-Myers syndrome, mental retardation, short stature, cryptorchidism, ATR-X syndrome, thalassemia, Fanconi anemia, Hodgkin disease, systemic lupus erythematous, SLE, rheumatoid arthritis, inflammatory bowel disease, graft versus host disease, nephrotic syndrome

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Contributor Information and Disclosures

Author

Joseph C Turbyville, MD, Fellow, Department of Allergy and Immunology, Walter Reed Army Medical Center
Joseph C Turbyville, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, and American College of Allergy, Asthma and Immunology
Disclosure: Nothing to disclose.

Coauthor(s)

Cecilia P Mikita, MD, MPH, Associate Program Director, Allergy-Immunology Fellowship, Chief, Clinical Services, Allergy-Immunology Clinic, Walter Reed Army Medical Center
Cecilia P Mikita, MD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, and Clinical Immunology Society
Disclosure: Nothing to disclose.

Mudra Kumar, MD, MBBS, MRCP, Associate Professor, Department of Pediatrics, University of South Florida College of Medicine
Mudra Kumar, MD, MBBS, MRCP is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Ann O'Neill Shigeoka, MD †, Former Clinical Associate Professor, Department of Pediatrics, Division of Immunology-Rheumatology, University of Utah School of Medicine
Ann O'Neill Shigeoka, MD † is a member of the following medical societies: American Federation for Medical Research, Clinical Immunology Society, Pediatric Infectious Diseases Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

John Wilson Georgitis, MD, Consulting Staff, Lafayette Allergy Services
John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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