Pallor - Case 10-3: 5-Month-Old Boy
I. History of Present Illness
A 5-month-old African-American boy presented with pallor, difficulty breathing,
and lethargy. He had been in his usual state of good health until 4 days before
admission, when he developed a fever to 39 to 39.5
°C with rhinorrhea. There was no coughing, vomiting, or diarrhea. The patient
otherwise seemed well at the time. Over the next few days, he developed
increased work of breathing with decreased appetite. One day before admission,
the mother noted that he seemed lethargic and irritable. Oral intake was
significantly decreased; the infant was taking in only 8 ounces rather than his
typical 48 ounces over the day. The patient was brought to the emergency
department. In retrospect, the father noted that the child
's abdomen seemed to be increasing in size and firmness over the last month, with
some tenderness.
II. Past Medical History
The infant was born at term after an uncomplicated pregnancy. He was taken home
on the second day of life. He had no known allergies. He did not take any
medications. He had received the appropriate immunizations for age, including
two doses of the heptavalent pneumococcal conjugate vaccine. The family history
was notable for sickle cell disease in a paternal cousin and reactive airways
disease, cervical cancer, and ovarian cancer in several maternal relatives. A
great uncle died of
“jaundice” at 3 years of age and a cousin at 10 years of age.
III. Physical Examination
T, 38.4°C; RR, 58/min; HR, 160 bpm; BP, 83/38 mm Hg
Weight, 5.7 kg
In general, the child was lethargic and responsive only to painful stimuli. He
also had severe respiratory distress. The anterior fontanel was sunken. The
pupils were equal, round, and reactive to light. The conjunctivae and oral
mucosae were pale. The lips were dry and cracked. There were white patches on
the buccal mucosa that were easily removed with scraping. There was shotty
anterior and posterior cervical adenopathy. The lungs were clear to
auscultation, but the child had mild grunting and flaring. There was a II/VI
systolic ejection murmur at the left lower sternal border. The abdomen was
firm, with a liver edge palpable 5 cm below the right costal margin. The spleen
was palpable at the level of the umbilicus. Bowel sounds were present. The
extremities were cool with delayed capillary refill (5 seconds). On neurologic
examination, the child localized pain but had decreased tone and diminished
spontaneous activity.
In the emergency department, the patient had an oxygen saturation of 94% in room
air. He received several normal saline boluses as well as sodium bicarbonate
and intravenous dextrose. Blood and urine cultures were obtained. Intravenous
cefotaxime was given for presumed sepsis.
IV. Diagnostic Studies
The complete blood count revealed the following: 14,100 WBCs/mm3 (2% band forms; 52% segmented neutrophils, 42% lymphocytes, 2% eosinophils, and
2% atypical lymphocytes); hemoglobin, 2.6 g/dL; 184,000 platelets/mm
3; MCV, 88 fL; RDW, 17.4. The total bilirubin was 4.6 mg/dL with an unconjugated
level of 3.8 mg/dL. Hepatic transaminases, were normal but the lactate
dehydrogenase level was 2,984 IU/L (normal range, 934 to 2,150 IU/L). The chest
radiograph was normal. There was no cardiomegaly, infiltrates, or mediastinal
masses.
V. Course of Illness
The peripheral blood smear (Fig. 10-1), in conjunction with other laboratory
findings, suggested the diagnosis.
Discussion: Case 10-3
I. Differential Diagnosis
This child came to the hospital with significant severe pallor of acute onset.
The child had had no medications or unusual exposures. It was clear that he was
critically ill. Despite the severe illness, the WBC and platelet counts were
normal. The most significant finding was the severe anemia. The increased
unconjugated bilirubin and lactate dehydrogenase levels suggested a hemolytic
process. Other causes of hemolytic anemia were considered, including
drug-associated hemolytic anemia, disorders of RBC membrane and cytostructure
(e.g., hereditary spherocytosis), abnormalities of RBC metabolism (e.g., G6PD
deficiency), as well as sepsis with disseminated intravascular coagulation. The
patient was given antibiotics to cover this last possibility. Parvovirus B19
infection can cause severe anemia but usually as a result of bone marrow
suppression rather than hemolysis.
Patients with a microangiopathic hemolytic anemia (e.g., hemolytic-uremic
syndrome, thrombotic thrombocytopenic purpura) usually have schistocytes rather
than spherocytes on peripheral blood smear. Both of these conditions usually
present with severe thrombocytopenia. A child of this age should be closely
examined for physical abnormalities seen with Diamond- Blackfan syndrome or
Fanconi anemia. Laboratory studies allowed differentiation of the diagnostic
possibilities.
II. Diagnosis
The peripheral blood smear revealed a nucleated RBC (erythroid progenitor cell)
prematurely released from the bone marrow into the peripheral circulation (Fig.
10-1). There were also many small spherocytes. Polychromasia was also noted,
because the bone marrow was releasing large numbers of reticulocytes and
nucleated RBCs to compensate for accelerated RBC destruction. A direct
antiglobulin (Coombs) test was positive, confirming the presence of antibodies
to circulating RBCs. Specifically, this test was positive for immunoglobulin G
(IgG) and negative for C3, consistent with the diagnosis of warm agglutinin
autoimmune hemolytic anemia (AHA).
The diagnosis is AHA of the warm agglutinin type.
III. Incidence and Epidemiology
AHA occurs as the result of binding of antibody, antibody and complement
complex, or complement to the RBC. The resulting immunologic reaction destroys
RBCs and causes anemia. Infectious agents, drugs, and other agents may
stimulate the process. Some autoimmune diseases, such as systemic lupus
erythematosus, may also generate anti-RBC antibodies and RBC destruction. The
true incidence of AHA is unknown, but is estimated to be 1 to 3 cases per
100,000 population per year. Acute AHA usually manifests in the first 4 years
of life.
IV. Clinical Manifestation
Children usually present with signs and symptoms of severe anemia. In younger
children, parents or other family members may note pallor or weakness. Older
children may complain of exercise intolerance or dizziness. Jaundice and
scleral icterus result from the recycling of unconjugated bilirubin released
from hemolyzed RBCs. Dark urine suggests hemoglobinuria. On examination, there
is usually mild or moderate splenic enlargement. Some patients present with
congestive heart failure related to the rapid development of anemia.
V. Diagnostic Approach
Complete blood count. The anemia of acute-onset AHA is usually significant. Most children have a
hemoglobin level of 4 to 7 g/dL. The MCV may be relatively normal, reflecting
the weighted average of small microspherocytes and large reticulocytes.
Erythrocyte agglutination within the sample tube may artifactually raise the
MCV on an automated counter. An elevated RDW may be a clue that several
different RBC populations are present, such as microspherocytes forming after
partial splenic ingestion and large reticulocytes. There is an increase in
reticulocytes once the bone marrow has a chance to respond. The WBC and
platelet counts are typically normal. Concurrent thrombocytopenia indicates
Evan
's syndrome, aplastic anemia, hemolytic-uremic syndrome, or other conditions,
rather than isolated autoimmune hemolytic anemia.
Direct antiglobulin test. A positive Coombs test confirms the suspected diagnosis. To establish a
diagnosis, there must be antibody and evidence of hemolysis. The RBC antibodies
that react at 37
°C (warm antibodies) are usually IgG and do not cause spontaneous agglutination
of cells unless Coombs antiserum is added. Cold antibodies are usually IgM and
react at 4
°C. These are considered complete antibodies, because no antiserum needs to be
added to cause the RBC destructive process. There are also cases of mixed-type
autoimmune responses.
Other studies. Other tests can provide evidence of an underlying hemolytic process: elevated
unconjugated bilirubin, elevated lactate dehydrogenase, decreased serum
haptoglobin, and urinalysis revealing blood on dipstick but no RBCs on
microscopy (hemoglobinuria). Hepatic transaminases should be normal.
VI. Treatment
The mainstay of treatment is corticosteroids, given at a dosage of 2 to 10 mg/kg
per day. In severely ill patients such as this child, the steroids should be
given intravenously. Otherwise, the care is supportive and includes replacement
therapy. Compatible cross-matching may not be possible, but if the patient is
in extremis blood type group O, Rh-negative blood should be used in aliquots
that are given slowly and in amounts sufficient to stabilize the cardiovascular
system. The patient should be adequately hydrated to avoid renal involvement.
Splenectomy may be necessary if steroid therapy fails.
VII. References
1. Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol 2002;69:258–271.
2. Flores G, Cunningham-Rundles C, Newland AC, et al. Efficacy of intravenous
immunoglobulin in the treatment of autoimmune hemolytic anemia: results in 73
patients.
Am J Hematol 1993;44:237–242.
3. Ware RE. Autoimmune hemolytic anemia. In: Nathan DG, Orkin SH, Ginsburg D,
et al., eds.
Nathan and Oski's hematology of infancy and childhood, 6th ed. Philadelphia: WB Saunders, 2003:521–559.
Pictures
Book Source Details
- Book Title: Pediatric Complaints and Diagnostic Dilemmas
- Author(s): Samir S Shah MD; Stephen Ludwig MD
- Year of Publication: 2003
- Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.
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Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.
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