A 15-year-old thalassemic patient with sudden onset of high fever (page 1/3)

Prepared by.... Patcharaporn Wongkaewpothong, M.D.
Janya Ananpiboonsri, M.D.
Thanyawee Puthanakit, M.D.
Virat Sirisanthana, M.D.
Patient: A 15-year-old girl with beta-thalassemia major s/p splenectomy
CC: High-grade fever 6 hours prior to admission
Present Illness:
6 hr PTA: She developed a sudden onset of high fever, headache and malaise. She had one episode of non mucous bloody diarrhea and non bilous vomitting.
Past Medical History:
> She had underlying beta thalassemia major. She had splenectomy when she was 4 year old..
> No prophylactic antibiotics and pneumococcal immunization.
> She received blood transfusion every month with regular iron chelation therapy.
> She has had 3 episodes of pneumonia that required hospitalization after the splenectomy.
Physical examination:
 

GA: A girl with normal consciousness, febrile
V/S: BT 39.2 C, PR 130/min, BP 120/50 mmHg, RR 36/min.
BW: 32 kg (< 3rd percentile)
HEENT: mild pale conjunctivae, mild icteric sclerae, no injected pharynx, tympanic membrane intact
LN: not palpable
Heart: regular rhythm, tachycardia, SEM gr.3/6 at apex
Lungs: clear, no adventitious sound
Abdomen: soft, no guarding, liver 8 cm below RCM
Ext: no edema, no skin rash, capillary refill < 2 seconds

Question 1: What is the diagnosis?
Diagnosis: Septicemia in asplenic patient
Discussion:
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Asplenic patients have increased risk for overwhelming, life-threatening infections, most commonly due to S.pneumoniae (50-90%), H.influenza, N.meningitidis, Samonella spp.
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The level of risk appears to correlate inversely with the amount of time a patient has had a functioning spleen. Overall, approximately 5% of children whose spleen are removed before the age of 4 years have significant infections, with a mortality rate of 30% to 60%.
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Risk of infection is greatest in the year after splenectomy (regardless of age) and continues to be significant in the next 7 to 10 years, after which risk is low but never as low as for patients with normal splenic function.
Pathogenesis
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The central role of the spleen are (1) mechanical clearance of antigen and foreign material, (2) synthesis of factors such as tuftsin, which enhance phagocytosis, and (3) coordination of interactions of the T-lymphocyte and B-lymphocyte response to antigens.
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The encapsulated organisms require antibody and complement for opsonization and clearance. However, in the absence of preformed antibody, the spleen is critical in clearing of the blood stream.
Clinical manifestations
Characteristics of overwhelming postsplenectomy sepsis include the sudden onset of fever and chills, vomiting and headache. The illness rapidly progresses to hypotensive shock and is commonly accompanied by disseminated intravascular coagulation

Question 2: What is the proper management?

Management of febrile illness in asplenic patient should include:
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Careful clinical evaluation
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Investigations (including hemoculture)
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Empirical antibiotic
Progression of the case Continue........... Next page

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