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20102009

ABSTRACTS

Proceedings of Sultan Qaboos University 
Clinical-Pathological Conferences 

2009–2010

SQU Med J, May 2011, Vol. 11, Iss. 2, pp. 301-303, Epub. 15th May 11

Pulmonary Sarcoidosis: One diagnosis, two different presentations
B Jayakrishnan,1 Fatma Ben Abid,1 Ana Maria A Serrano,2 Aisha Al-Hamdani3
Departments of 1Medicine, 2Family Medicine & Public Health and 3Pathology, Sultan Qaboos University Hospital, Muscat, Oman. Email: drjayakrish@hotmail.com

Even 140 years after the initial description, the aetiology of sarcoidosis, a multisystem disorder, remains elusive, the diagnostic tests 
remain largely nonspecific and treatment produces inconsistent results. 

Case One: A 69 year-old Omani female with hypertension, diabetes mellitus, depression and blindness was referred for evaluation of 
bilateral opacities on a chest radiograph. She had a three-month history of dry cough, exertional breathlessness, chest discomfort and 
weight loss. She denied any history of fever, night sweats, haemoptysis or recent travel. Clinical examination revealed a few small lymph 
nodes in the axillae. Basic blood tests, including serum calcium, were normal. The serum angiotensin converting enzyme (SACE) level 
was also normal. The sputum smear was negative for acid fast bacilli (AFB) by direct smear. The Mantoux test was negative. The chest 
radiograph showed bilateral parenchymal infiltrates. A computed tomography (CT) of the chest revealed bilateral extensive perihilar 
airspace consolidation with significant mediastinal lymph nodes. Spirometry results were as follows: forced expiratory volume 1 (FEV1) 
- 0.66 L (37%), forced vital capacity (FVC) - 1.01L (47%), FEV1/FVC - 65%.  A bronchoscopy showed hyperaemic mucosa with diffuse 
granular changes and a few nodular lesions. An endobronchial biopsy revealed features of non-caseating granuloma confirming a 
diagnosis of sarcoidosis.

Case Two: A 42 year-old Omani female presented with a dry cough, generalised itching and extreme tiredness of 6 months duration. 
On examination, there were well demarcated hyperpigmented brown nodular skin lesions over the breast, abdomen, lower back and 
both thighs. Some showed central ulceration with healing and post-inflammatory hypopigmented and hyperpigmented patches and 
superficial mild depressed scars. Routine blood tests, including serum calcium and SACE level, were normal. The Mantoux test was 
positive, 24 mm. The chest radiograph and computed tomography (CT) scan revealed bilateral hilar and mediastinal lymphadenopathy. 
Pulmonary function test results were as follows: FEV1- 1.87 L (78%), FVC - 2.29 L (81%), FEV1/FVC - 82%,  carbon monoxide diffusing 
capacity (DLCO) -75%, and carbon monoxide transfer coefficient (KCO) - 96%. The bronchoscopy did not show any abnormality; the 
bronchoalveolar lavage and sputum were negative for AFB by stain and culture. Skin lesions were biopsied, as well as a mediastinoscopy 
and mediastinal lymph node biopsy. Both skin and lymph node biopsies showed non-caseating granulomas suggestive of sarcoidosis.

Both the patients received prednisolone leading to symptomatic and radiological improvement. Since the Mantoux test was positive, the 
second patient received anti tuberculosis treatment prior to the initiation of steroids. 

Despite advances in the knowledge of the immunopathogenesis of sarcoidosis, no single aetiologic agent or genetic locus has been 
clearly identified in the development of the disease.1 The disease varies in incidence and presentation among geographical regions.2 Any 
organ can be involved, the most commonly affected sites being the lungs (approximately 90% of patients), lymph nodes, skin, eyes, and 
the liver. Cutaneous involvement is common presenting as macular, nodular, maculopapular, ulcerative or plaque like lesions; it can also 
present as verrucous, ichthyosiform, hypomelanotic, or psoriasiform lesions.3 They can be divided into specific lesions and nonspecific 
lesions based on the characteristics and histology,4 the classic specific and nonspecific lesions being Lupus pernio and Erythema 
nodosum respectively. Spontaneous remission is common in sarcoidosis so not all patients require treatment. Generally, treatment is 
indicated when there are debilitating symptoms or evidence of significant or progressive end-organ damage. Glucocorticoids are the 
commonest treatment agents for pulmonary sarcoidosis. Refractory disease may require other agents such as immunosuppressive, 
cytotoxic, antimalarial drugs or anti-tumour necrosis factor (TNF) antibodies.



302 | SQU Medical Journal, May 2011, Volume 11, Issue 2

Proceedings of Sultan Qaboos University Clinical-Pathological Conferences 

r e f e r e n c e s

1. Baughman RP, Culver DA, Judson MA. Concise Review of Pulmonary Sarcoidosis. Am J Respir Crit Care Med 2010, Oct 29.  
 [Epub ahead of print]

2. Behbehani N, Jayakrishnan B, Khadadah M, Farrah Y, Hawa H. Clinical Presentation of Sarcoidosis in a Mixed Population in  
 the Middle East. Respir Med. 2007; 101:2284–8.

3. Macrophage/Monocyte disorders In: James WD, Berger TG, Elston DM, Eds. Andrews' Diseases of skin, Clinical Dermatology.  
 10th ed. Philadelphia: W.B. Saunders, 2005. Pp. 708–14.

4. Rose AS, Tielker MA, Knox KS. Hepatic, ocular, and cutaneous sarcoidosis. Clin Chest Med 2008; 29:509–24.

These cases were presented at the Sultan Qaboos University Clinical-Pathological Conference on 7th April 2010 with the title “To Be or 
not To Be…”. The title represents the uncertainties in the aetiology, diagnosis and the treatment of sarcoidosis as well as the diagnostic 
confusion in differentiating it from tuberculosis.

Girl with T-Cell Leukaemia and Severe Central Nervous System Involvement
Yasser Wali,1 *Mathew Zachariah2 Ismail El Beshlawi,2 Nancy Reyes2 and Nasser S. Al-Hajari2
1Department of Child Health, College of Medicine & Health Science Sultan Qaboos University, Muscat, Oman. Departments of 1Child Health, 2Nursing, 3Physiotherapy, 
Sulltan Qaboos University Hospital, Muscat, Oman. Email: mathewz@squ.edu.om 

Remarkable advances have been achieved in the treatment of acute lymphoblastic leukemia (ALL) in children in the last decades. 
However, still 20% of children ultimately relapse, and the cure rate after relapse is only 25% to 40%. T-cell acute lymphoblastic leukemia 
(T-ALL) accounts for 10% to 15% of newly diagnosed cases of childhood acute lymphoblastic leukemia (ALL). Age, total white blood cell 
count at diagnosis, immunophenotype, chromosomal aberrations, speed of treatment response, minimal residual disease, are all very 
important factors in determining the prognosis of the diseases. An eight year-old Omani girl was admitted to Sultan Qaboos University 
Hospital (SQUH) referred from a peripheral hospital with fever and poor school performance of more than one month’s duration. 
The child had developed convulsions, septic shock and was ventilated and was therefore transferred to SQUH as a suspected case of 
leukaemia. In SQUH she was labelled as T-Cell leukaemia with central nervous system (CNS) involvement and was completely aphasic. 
Despite repeated magnetic resonance imaging (MRI) brain scans that confirmed white matter changes and demyelination initially and 
widely extended on repeated imaging, her cerebrospinal fluid (CSF) was repeatedly clear. After successful induction with a POG 9404 
T3 protocol, she had a severe episode of febrile neutropenia that rapidly progressed to septic shock, deteriorating neurological status 
and respiratory distress. She had recurrent episodes of febrile neutropenia, sepsis with ELBS E.Col , coagulase negative Staphylococcus 
and Candida. Her condition deteriorated with severe mucositis and respiratory distress which needed an emergency tracheostomy 
and later she was put on mechanical ventilation. She remained in very critical situation for 4 weeks and she was labelled as DNR (Do 
Not Resuscitate) status. Her chemotherapy was withheld for two and a half months due to her grave clinical condition. Of interest, 
she maintained her remission status on a repeat bone marrow aspiration; however; she had extensive haemophagocytosis (secondary 
haemophagocytic lymphohistiocytosis [HLH]) that needed to be treated with partial induction for 6 weeks (HLH 2004). After all these 
stormy events child ended up in a devastating state with severe weight loss (skin on bones), aphasia, and inability to walk. With extensive 
nursing support and physiotherapy, she improved remarkably, started to gain weight, her speech recovered and the tracheostomy was 
closed. Her MRI brain scan was almost normal. After one year of an uneventful course of maintenance chemotherapy, she suddenly 
developed facial palsy. Other CNS examinations were normal and the CSF was still clear. Unfortunately, her bone marrow showed 80% 
blasts of the initial phenotype confirming a combined bone marrow and CNS relapse. The brain images were obviously normal. She was 
started on UK ALL relapse protocol and planned for early bone marrow transplantation. During the induction phase she had another 
episode of febrile neutropenia, herpes simplex virus lesions that further deteriorated and progressed to septic shock.  After a long severe 
prolonged course she expired. Everyone has got only one life; however, in our case she was labelled as DNR once and all the treating 
team members lost hope that she would survive. Such a courageous young girl made it through and came out of this tragedy learning 
again how to talk and walk.

This case was presented at the Sultan Qaboos University Clinical-Pathological Conference on 5th May 2010 with the title“You Only Live 
Twice”

Between the Loins and the Ribs - A case of a germ cell tumour
Ibtisam H. Al-Shuaili,1 Aisha Al-Hamdani,2 Sinan Al-Azzawi,3 Ibrahim Inuwa4 and Ikram A Burney1
Departments of 1Medicine, 2Pathology, 3Radiology & Molecular Imaging, Sultan Qaboos University Hopsital, Muscat, Oman; 4Department of Human & Clinical 
Anatomy, College of Medicine & Health Science Sultan Qaboos University, Muscat, Oman. Email: ibtisamsh@hotmail.com

A 21 year-old boy, previously well, presented at Sultan Qaboos University Hospital, Oman, with a 2-month history of low grade fever, 
malaise, weight loss of about 3–4 kg, and haemoptysis. There was no history of shortness of breath, palpitation, recent travel, or contact 
with patients suspected to have tuberculosis or HIV. The physical examination was unremarkable apart from a dull percussion note with 
reduce breath sounds on left side of the chest. Vital signs were temperature 38 ºC, pulse rate 128 bpm, respiratory rate 14/min, blood 
pressure 97/50 mmHg. A blood investigation revealed mild anaemia with an Hb of 9 g/dl, normochromic normocyctic, and normal white 
cell and platelet counts. The coagulation profile, urea and electrolytes, liver function tests and the bone profile were all within the normal 
limits. A chest X-ray was done and reported a large mediastinal mass. A differential diagnosis of a germ cell tumour was entertained and 
a serum alfa-fetoprotein (AFP) was found to be markedly elevated at 20,142 KIU/L, whereas, serum lactate dehydrogenase (LDH) was 



Abstracts | 303

2009–2010

minimally elevated to 698 U/L (up to 248 IU/L). Serum and urine β-human chorionic gonadotropin (HCG) were within the normal limits. 
An ultrasound scan of the testis was unremarkable, and a computed tomography scan of chest [Figure 1a] showed a large ill-defined 
lobulated anterior mediastinal mass in the superior mediastinum, extending into the left hemithorax. The mass was seen displacing the 
mediastinal vascular structures to the right and posteriorly, with significant compression of the left main pulmonary artery and possible 
infiltration of the main pulmonary artery. The left upper lobe bronchus was collapsed with loss of volume of the left upper lobe. The liver 
was found to be enlarged and showed numerous focal lesions predominantly in the right lobe with central necrosis and thick enhancing 
rims [Figure 1b]. A biopsy revealed an extensively necrotic poorly differentiated malignant neoplasm with the immunohistochemical 
profiles in keeping with mixed germ cell tumor. The patient was treated with combination chemotherapy consisting of bleomycin, 
etoposide and cisplatin (BEP). The tumour marker response is shown in Figure 2. Because of a lack of marker normalisation after 
three cycles of the combination chemotherapy, the treatment was changed to a combination of vinblatine, ifosfamide and cisplatin, of 

which he received four cycles. The treatment was further changed 
to third line chemotherapy consisting of gemcitabine and oxaliplatin 
because of lack of marker remission. Owing to no further marker 
decline, the patient was referred for a positron emission tomography 
(PET) scan, which revealed increased uptake in the mediastinum, 
but not in the liver. The patient underwent thoracotomy with 
resection of the residual mass lesion, the histopathology of which 
showed mainly necrosis. Following the thoracotomy, the alpha-
fetoprotein (AFP) dropped to normal limits, and the patient 
has been in continuous complete remission since March 2008. 
 
This case was presented at the Sultan Qaboos University Clinical-
Pathological Conference in DATE? April 2009 with the title “Between 
the Loins and the Ribs”. 

 
Figure 2: Alpha-fetoprotein (AFP) levels in response 
to treatment and over time. The X-axis shows time 
in weeks, and the Y axis shows the level of AFP on a 
logarithmic scale. BEP = bleomycin, etoposide and 
cisplatin; VeIP = vinblastine, ifosfamide and cisplatin; 
GemOx = gemcitabine and oxaliplatin.

Figure 1: Computed tomography (CT) scan of chest (1a) and the upper abdomen (1b), showing disease in the 
mediastinum and the liver before treatment. Figure 1c shows the CT scan following thoracotomy, resection of the 
residual mass.