Table of Contents
Patients, Families and Friends
Health Care Providers
|Specific Problems: Sequestration - Spleen or Liver|
|Health Care Providers - Problem Oriented Clinical Guidelines|
by Lewis Hsu, MD, PhD
Edited by James Eckman, M.D. and Allan Platt, PA-C
Splenic sequestration episodes occur in highest frequency during the first five years of life in children with sickle cell anemia (HbSS). Splenic sequestration occurs at any age in individuals with the other sickle syndromes. In all sickle syndromes, sequestration may be seen primarily in the liver in older individuals. This complication is characterized by onset of life-threatening anemia with rapid enlargement of the spleen and/or liver with a high reticulocyte count. In older individuals with primarily hepatic sequestration, there may be rapid deterioration of hepatic function, rhabdomyolysis, and renal insufficiency.(See multi-organ system failure chapter) Treatment of the acute episode requires early recognition, careful monitoring, aggressive transfusion support, and occasional emergent splenectomy. Episodes tend to be recurrent so many advocate splenectomy or chronic transfusion.
Worsening severity of anemia (falling below baseline for that patient) can be a sign of developing life-threatening complications such as aplastic crisis and splenic sequestration.
General guidelines suggesting a need for evaluation include.
Present Illness. Patient may be asymptomatic or have symptoms of acute lethargy, loss of appetite, increased weakness, fatigue, or dyspnea on exertion. History of recent infections, change in jaundice, increased pallor, increase in abdominal girth, bruising, petechiae, change in urine color, dizziness, syncope, and present medications. Review spleen stick (See parent education).
Review of symptoms. Specifically exclude symptoms of infection, left or right upper quadrant discomfort, early satiety, skin rash, gastrointestinal symptoms, symptoms of pregnancy such as missed menstrual period, morning sickness, breast tenderness.
Past Medical History. Note previous episodes of severe anemia, transfusion history, history of splenomegaly and potential sources of blood loss such as ulcer disease.
- Vital signs. Temperature, respiratory rate, supine/upright blood pressure and pulse.
- HEENT. Look for increased jaundice, retinal hemorrhages, and oral petechiae.
- Neck. Lymphadenopathy.
- Chest. Bibasilar rales, signs of consolidation.
- Heart. Neck veins, S3, increased murmur, pedal edema.
- Abdomen. Increase in size of liver or spleen,
- Extremities. Edema, ecchymosis, petechiae.
- Neuro. Mental status changes. Irritability in infant or toddler.
- Minimum Lab. CBC with differential, reticulocyte & platelet count, Chem profile including electrolytes, AST, ALT, bilirubin, alk ptase, LDH, BUN, Creat, Urinalysis
- Aplastic Crisis. Hemoglobin drop of more than 1 gm% or absolute value of less than 5 gm% with an absolute reticulocyte count of less than 10,000. True aplastic crisis with very low reticulocyte count and only anemia is usually seen in children and associated with infection by the parvovirus B19. Relative aplastic crisis with a reticulocyte less than 100,000 and a falling hemoglobin, may be associated with viral or bacterial infection, pain episodes, or occur de novo. White count and platelet count may also be low.
- Splenic Sequestration Crisis. Hemoglobin drop of more than 1 gm% or absolute value of less than 5 gm% with reticulocyte count of more than 100,000 with rapidly enlarging spleen and/or liver. There is often increase in indirect bilirubin AST, alk ptase, and LDH over baseline. In adults, there may be evidence of hepatic dysfunction, renal insufficiency, and rhabdomyolysis. Splenic sequestration is more common in children less than three and is a significant cause of morbidity and mortality. Splenic sequestration can be seen at any age in patients with Hb SC or S beta-thal. In adults with sickle cell anemia, sequestration of the liver is more common.
- Bleeding. Hemoglobin drop with orthostatic hypotension, hematemesis, melena, or guaiac positive stool suggests acute bleeding as a cause of the more severe anemia.
- Anemia of Renal Insufficiency. Increasing anemia is seen with an elevated serum creatinine or standard creatinine clearance of less than 50 ml/min/1.76m2. This problem is becoming more common as patients are living longer. The etiology may be a relative erythropoietin deficiency caused by the renal damage.
Criteria for Admission
- Aplastic crisis or sequestration crisis both require immediate admission, preferably to an intensive care unit.
- Any patient with an unstable hemoglobin level of less than 6 gm% should be admitted.
- Almost all patients with drop in hemoglobin of over 1 gm% or hematocrit of 3 vol% and. heart failure, pain crisis, GI or GU bleeding, orthostatic drop in blood pressure (10 mm Hg) or increase in pulse (20/min), or documented infection require admission to the hospital for evaluation and treatment. Note that young children may manifest congestive heart failure as hepatosplenomegaly.
- Splenic Sequestration Crisis. Admission to a unit with a high level of care is indicated for patients with these syndromes. Frequent monitoring of vital signs, spleen and liver size, hemoglobin, liver tests, clotting studies, and renal function are indicated in the severe episodes. These syndromes have a very high mortality if not managed aggressively. Immediate transfusion with packed red cells is almost always indicated to maintain a hemoglobin level of no higher than 9 to 10 gms%. This is the only specific treatment available for hyperhemolytic episodes where only the liver is involved. Higher hemoglobin levels should be avoided because the hemoglobin may increase further as the episode resolves ("auto-transfusion," mobilizing RBC from the sequestering organ), causing problems with viscosity if the percent of Hb A is less than 50%. Other support measures, including fluids, electrolytes, vitamin K, and antibiotics, should be provided based on observed abnormalities.
- Splenic Sequestration - Children over Five and Adults. Many advocate splenectomy in children over five with one episode of sequestration because of a very high recurrence rate. Others will delay splenectomy until the second episode. Pneumococcal vaccine and meningococcal vaccine are indicated before surgery. Prophylactic penicillin is indicated after surgery. Individuals that do not have their spleens removed should be maintained on chronic transfusions for a presently unknown period of time.
- Splenic Sequestration - Children under Five. Children under two are usually treated with chronic transfusion every 3 to 4 weeks to maintain the hemoglobin at about 10 gm%. These are continued until the child is two. They are then stopped and splenectomy performed for recurrences after acute management. Children with a severe episode of splenic sequestration (e.g. requiring transfusion) between three and five should be considered for an elective splenectomy. Recurrences on transfusion or alloimmunization should prompt splenectomy in most cases.
- Renal Insufficiency. Patients with an erythropoietin level of less than 200 u/ml may respond to exogenous erythropoietin administration. Doses from 4000 to 10,000 units three times a week are often effective.
Patients and parents must be taught the early detection of splenic sequestration at diagnosis. Signs and symptoms of increasing anemia or hemolysis must be delineated. Hemoglobin levels, reticulocyte counts, indirect bilirubin, and LDH should be determined on a regular basis during health maintenance to establish a baseline for each patient. Hemoglobin and reticulocyte levels must be checked when the patient presents with any complication.
Patient and Parent Education
Education about diet, compliance with medications, and vitamins is important. The parents of infants need to be taught the signs of increasing anemia (pallor, lethargy, irritability, poor appetite, and increased jaundice). They need to learn how to assess spleen size The use of a wooden tongue depressor as a "spleen measuring stick" provides an accurate way of assessing and recording spleen size at home and in the clinic. In small children, one end can be placed on the left nipple and the distance to the spleen tip recorded in ink and dated. In older children, the distance from the ribs to the spleen tip in the left nipple line is recorded. Limits can be set by drawing red lines in ink and instructing the parent to being the child for immediate care if the spleen is increased to the line. Parents should be encouraged to check the spleen size on a regular basis and whenever the child appears ill. Names and phone numbers of individuals that need to be contacted can be written on the back of the spleen stick. The need to bring the spleen stick with them to every follow-up and emergency visit needs strong reinforcement.
Figure 6-1. Measurement of spleen size with a spleen stick.
Risks and benefits of transfusions and surgery need to be explained in patients that require these forms of therapy. Alternative forms of therapy are often available. The risks and detection of infection needs to be emphasized if the spleen is removed. Emergent splenectomy for acute splenic sequestration is not prudent, because the splenic sequestration is often triggered by infection or other problem that would increase the risk of perioperative complications.
CHRONIC SPLENOMEGALY - If splenomegaly is asymptomatic, then transfusions and surgery are not indicated. It is prudent to note the size of the spleen and ask family to monitor the size with a spleen stick, and often the spleen will gradually involute over months to years without causing acute splenic sequestration symptoms. However, chronic symptoms of hypersplenism may be an indication for surgery.
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