Common variable immune deficiency syndrome

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CASE REPORT Common variable immune deficiency syndrome Ahmed A. El-Masry, Ibrahim Dwedar, Hesham A. Abdel-Halim * , Riham Hazem Faculty of Medicine, Ain Shams University, Egypt Received 26 December 2013; accepted 5 January 2014 KEYWORDS Common variable immune deficiency; IgG; IgM; Pneumonia Abstract A male patient 40 years old, non smoker, presented with fever, cough and expectoration of greenish sputum and diarrhea of 1 week duration. The condition started 3 years ago, by cough and expectoration of about cup/day of greenish sputum, not related to posture, along with fever up to 39 °C with loss of weight about 12 kg in one month and associated with diarrhea and mucus shedding. The patient sought medical advice and received empirical antibiotics and symptomatic treatments with partial clinical improvement. The patient showed multiple relapses of same respi- ratory and gastro-enterology symptoms every 3–4 weeks with 4 hospital admissions. CT-chest was done and revealed emphysematous changes with basal inflammatory reaction (Fig. 1); sputum workup showed no acid fast bacilli and growth of normal flora, fasting blood glucose was 102 mg/ dl. A second CT-chest was done after one year and showed right sided pneumonic consolidation, bilateral pneumonic reaction with multiple mediastinal lymphadenopathy, bilateral pleural thicken- ing and right encysted pleural effusion (Fig. 2). ª 2014 Production and hosting by Elsevier B.V. on behalf of The Egyptian Society of Chest Diseases and Tuberculosis. Fibro-optic bronchoscopy was done and showed mucosal ulceration of the right lower lung segments with purulent dis- charge from that segments, BAL C&S showed gram stain mixed g-ve bacilli (proteus and pseudomonas), after 4 months, sputum C&S showed a growth of candida. There was no past or family history of relevant significance (Figs. 1 and 2). Physical examination There was a fever of 38–39.5 °C during the first 9 days of hos- pital course, chest clinical examination showed inspiratory coarse crackles all over chest. Laboratory data ABG on room air was (PH = 7.47, PaCO 2 = 28 mmHg, PO 2 = 73 mmHg, HCO 3 22 mEq/l, ABE 3.2, SO 2 = 96%), ECG showed sinus tachycardia, CBC revealed (WBC 22.200 (Neutrophils 82.2%), Hb 9.1 (normocytic normochromic), platelets (446.000), hypokalemia (K 2.0) which was corrected by IV potassium, fasting blood glucose, liver, kidney and coag- ulation profiles were normal. Sputum workup revealed no acid fast bacilli nor any pathogenic organisms, stool analysis showed entameba histolytica cysts, stool C&S revealed normal * Corresponding author. Tel.: +20 1224474750. E-mail address: [email protected] (H.A. Abdel-Halim). Peer review under responsibility of The Egyptian Society of Chest Diseases and Tuberculosis. Production and hosting by Elsevier Egyptian Journal of Chest Diseases and Tuberculosis (2014) xxx, xxxxxx The Egyptian Society of Chest Diseases and Tuberculosis Egyptian Journal of Chest Diseases and Tuberculosis www.elsevier.com/locate/ejcdt www.sciencedirect.com Please cite this article in press as: A.A. El-Masry et al., Common variable immune deficiency syndrome, Egypt. J. Chest Dis. Tuberc. (2014), http://dx.doi.org/10.1016/j.ejcdt.2014.01.002 0422-7638 ª 2014 Production and hosting by Elsevier B.V. on behalf of The Egyptian Society of Chest Diseases and Tuberculosis. http://dx.doi.org/10.1016/j.ejcdt.2014.01.002

Transcript of Common variable immune deficiency syndrome

Page 1: Common variable immune deficiency syndrome

Egyptian Journal of Chest Diseases and Tuberculosis (2014) xxx, xxx–xxx

The Egyptian Society of Chest Diseases and Tuberculosis

Egyptian Journal of Chest Diseases and Tuberculosis

www.elsevier.com/locate/ejcdtwww.sciencedirect.com

CASE REPORT

Common variable immune deficiency syndrome

Ahmed A. El-Masry, Ibrahim Dwedar, Hesham A. Abdel-Halim *, Riham Hazem

Faculty of Medicine, Ain Shams University, Egypt

Received 26 December 2013; accepted 5 January 2014

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Common variable immune

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Pneumonia

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Abstract A male patient 40 years old, non smoker, presented with fever, cough and expectoration

of greenish sputum and diarrhea of 1 week duration. The condition started 3 years ago, by cough

and expectoration of about cup/day of greenish sputum, not related to posture, along with fever up

to 39 �C with loss of weight about 12 kg in one month and associated with diarrhea and mucus

shedding. The patient sought medical advice and received empirical antibiotics and symptomatic

treatments with partial clinical improvement. The patient showed multiple relapses of same respi-

ratory and gastro-enterology symptoms every 3–4 weeks with 4 hospital admissions. CT-chest

was done and revealed emphysematous changes with basal inflammatory reaction (Fig. 1); sputum

workup showed no acid fast bacilli and growth of normal flora, fasting blood glucose was 102 mg/

dl. A second CT-chest was done after one year and showed right sided pneumonic consolidation,

bilateral pneumonic reaction with multiple mediastinal lymphadenopathy, bilateral pleural thicken-

ing and right encysted pleural effusion (Fig. 2).ª 2014 Production and hosting by Elsevier B.V. on behalf of The Egyptian Society of Chest Diseases and

Tuberculosis.

Fibro-optic bronchoscopy was done and showed mucosalulceration of the right lower lung segments with purulent dis-charge from that segments, BAL C&S showed gram stain

mixed g-ve bacilli (proteus and pseudomonas), after 4 months,sputum C&S showed a growth of candida. There was no pastor family history of relevant significance (Figs. 1 and 2).

4474750.om (H.A. Abdel-Halim).

e Egyptian Society of Chest

g by Elsevier

.A. El-Masry et al., Common0.1016/j.ejcdt.2014.01.002

ng by Elsevier B.V. on behalf of T

.002

Physical examination

There was a fever of 38–39.5 �C during the first 9 days of hos-

pital course, chest clinical examination showed inspiratorycoarse crackles all over chest.

Laboratory data

ABG on room air was (PH = 7.47, PaCO2 = 28 mmHg,PO2 = 73 mmHg, HCO3 22 mEq/l, ABE �3.2, SO2 = 96%),

ECG showed sinus tachycardia, CBC revealed (WBC 22.200(Neutrophils 82.2%), Hb 9.1 (normocytic normochromic),platelets (446.000), hypokalemia (K 2.0) which was correctedby IV potassium, fasting blood glucose, liver, kidney and coag-

ulation profiles were normal. Sputum workup revealed no acidfast bacilli nor any pathogenic organisms, stool analysisshowed entameba histolytica cysts, stool C&S revealed normal

variable immune deficiency syndrome, Egypt. J. Chest Dis.

he Egyptian Society of Chest Diseases and Tuberculosis.

Page 2: Common variable immune deficiency syndrome

Figure 1 CT-chest showing emphysematous changes with basal

inflammatory reaction.

Figure 2 CT-chest showing Right sided pneumonic consolida-

tion, bilateral pneumonic reaction with multiple mediastinal

lymphadenopathy, bilateral pleural thickening and right encysted

pleural effusion.

2 A.A. El-Masry et al.

flora. Colonoscopy was done up to cecum, examination re-

vealed normal mucosa and vascular pattern with no evidenceof ulcers, polyps or masses. ESR-1st hr 30, CRP 24, HIV-Ab, HBsAg, and HCV-Ab were negative. IGg < 50 mg/dl

(N. 658-1837) and IGA was 36 mg/dl (N. 71-360). Chest radio-graph revealed bilateral basal hetrogenous opacity (pneumo-nia), CT-chest showed Bilateral basal lung consolidative

patches involving the lingual and right middle lobe and Bilat-eral minimal pleural effusion and thickening, pelvi-abdominalU.S. showed calcular gall bladder and mild splenomegaly,Echocardiogram revealed normal LV internal dimensions

and normal systolic function, with estimated EF = 62%.

Please cite this article in press as: A.A. El-Masry et al., CommonTuberc. (2014), http://dx.doi.org/10.1016/j.ejcdt.2014.01.002

Immunology consultation suggested the diagnosis of ‘‘com-mon variable immune deficiency’’ and adviced prophylactictherapy in the form of long acting penicillin/2 weeks, SeptrinDS tab twice daily for 15 days, oral Ketrax 3tab every other

day for 15 days and follow up in outpatient clinic.Other tests recommended including C3, C4, Circulating im-

mune complex, and Immunefixation, IgM, IgE titers.

Conflict of interest

None declared.

variable immune deficiency syndrome, Egypt. J. Chest Dis.