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Case presentation Thalassemia Center. A 14-year old Omani girl Known case of Beta-Thalassemia Major Diagnosed at the age of 6 months. On regular transfusion every 4 weeks since that time. First visit to Thalassemia centre at the age of 9 years old. . Findings on 1 st presentation:

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Case presentationThalassemia Center


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  • A 14-year old Omani girl

  • Known case of Beta-Thalassemia Major

  • Diagnosed at the age of 6 months.

  • On regular transfusion every 4 weeks since that time.

  • First visit to Thalassemia centre at the age of 9 years old.


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Findings on 1st presentation:

  • Mild thalassemic features.

  • Severe growth retardation (below 3rd centile).

  • Liver: 8cm.

  • Spleen: 14 cm.

  • Hb: 6.8 g/dl.

  • S. Ferritin: 8600 ug/l

  • HCV RNA +ve


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Chelation history:

  • Not on any chelation therapy till the age of 9 years.

  • Desfral started at the age of 9 years

  • On Exjade since November 2006

    ( 500mg, 20 mg/kg daily )


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Family History:

23 y

22 y

16 y

16 y

14 y

9 y

6 y


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On Examination:

  • Good general condition

  • Vital signs are stable

  • Growth parameters are below 3rd centile

  • Pallor: ++

  • No jaundice

  • Abdomen: spleen 4 cm and liver 3 cm BCM.

  • CVS: normal.

  • Chest: clear

  • CNS: grossly intact.


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Investigations:

  • B gene: IVS 1-5 (G C) /CD 30 (G C)

  • One Alpha gene deletion.

  • HCV RNA turned –ve in Nov 2007 (no treatment).

  • EF was 60% in 2006.

  • Euglycemic, normal thyroid & parathyroid functions.


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Baseline investigations done before starting Exjade:

  • Serum ferritin: 2992 ug/l

  • Serum creatinine: 0.4 mg/dl

  • SGPT: 168 IU/L / SGOT: 91 IU/L

  • IGG: 30.3/ IGA: 3.2/ IGM: 3.5 (in 2004)

  • Anti ds DNA: -ve (in 2004)

  • ANF: +ve 1/1000 speckled (in 2004)

  • RF: +ve (in 2004)

  • Anti smooth muscle Ab: +ve (in 2004)


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Exjade started

HCV RNA –ve



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Definition

  • A disorder characterized by fever coinciding with the administration of the drug and disappearing after the discontinuation of the drug, when no other cause for the fever is evident after a careful physical examination and laboratory investigation


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Mechanisms

  • Hypersensitivity reactions

  • Altered thermoregulatory mechanisms

  • Directly related to administration of the drug

  • Direct extension of the pharmacologic action of the drug

  • Idiosyncratic reactions


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1. Hypersensitivity reactions:

  • Due to: 1. Ab-Ag complexes

    2. T-cell immune responce

  • Fever appears several days to three weeks after the drug has been started, but the lag time can be as long as several years.

  • Fever can arise within hours of a rechallenge, in a previously sensitized patient.


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  • Fever may the sole manifestation of a hypersensitivity reaction.

  • Hepatic, renal or pulmonary involvement, rash, mucosal ulceration, and hematologic abnormalities are not uncommon.

  • Withdrawal of the offending drug usually results in disappearance of the fever within 72 to 96 hours which helps to confirm the diagnosis.


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2. Altered thermoregulatory mechanisms: reaction.

  • Regulated by the anterior hypothalamus.

  • Fever is caused by releasing “Endogenous Pyrogens” (e.g: IL alpha & beta, TNF and Interferon alpha).

  • Drugs that can alter these Thermoregulatory mechanisms:

  • Exogenous thyroid hormone

  • Anticholinergics (e.g: TCA)

  • Sympathomimetics (e.g: amphetamines)


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3. Directly related to administration of the drugs reaction. :

  • Contamination of the administrated drug with endotoxin or other exogenous pyrogens.

  • Phlebitis, local inflammation and/or sterile abscesses can occur at sites of injection.


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4. Fever as an extension of the pharmacologic effect of a drug:

  • Fever observed following chemotherapy for various solid tumors, lymphomas, and leukemias due to release of different cytokines from cell necrosis and lysis.


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  • Fever starts drug:two to three days after chemotherapy and may last for one week or more.

  • This early febrile response usually can be distinguished from febrile neutropenia which rarely develops before the second week after chemotherapy.


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5. Fever due to an idiosyncratic reaction: drug:

  • These reactions include unpredictable syndromes and genetic disorders, and there is some overlap with hypersensitivity phenomena.

  • Malignant hyperthermia

  • Neuroleptic malignant syndrome (NMS)

  • Serotonin syndrome

  • Glucose-6-phosphate dehydrogenase deficiency:


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GENERAL CLINICAL ISSUES drug:

  • Drug fever is usually a diagnosis of exclusion.

  • The first assumption of most clinicians is that fever is due to infection, which may not always be easy to exclude.

  • Connective tissue diseases or malignancy are also often difficult to exclude.

  • Absence of the rash does not exclude Drug Fever as it is only present in 18% of cases.


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Fever patterns: drug:

  • The onset is in about eight days, but varies from less than 24 hours to many months.

  • fever may vary from a low-grade fever to high grade “hectic” fever with chills and rigors.

  • Patient’s condition may vary from severely ill to surprisingly well.

  • Relative bradycardia occurs in only about 10% of cases.


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Laboratory investigations: drug:

  • WBC can be elevated with eosinophilia and occurs in less than 20% of cases.

  • ESR is usually increased but this is a nonspecific finding.

  • Unexplained disturbance of liver function and/or renal impairment can provide clues to the diagnosis (e.g: interstitial nephritis caused by beta lactams).


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Cessation of the drug(s): drug:

  • The only real way to know if a patient has a drug fever in the majority of patients is by stopping the drug(s).

  • The usual clinical approach is to discontinue the most probable offending drug first, followed by cessation of other drugs if fever persists.

  • In most cases, resolution of drug fever will occur within 72 to 96 hours of discontinuing the offending drug.


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Rechallenge: drug:

  • Rechallenge with the offending drug can confirm the diagnosis if fever recurs.

  • Rechallenge is usually safe, particularly when the initial febrile illness is mild, but unexpected events can arise.

  • In clinical practice, rechallenge is not often performed.


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Thank you drug:


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