Haematology Watch


 Haematology Watch, Vol.4 , Issue 1.

 CASE REPORT

CASE REPORT

Mehmood



A 15 years old male patient presented from Jhelum with a chief complaint of swollen left jaw for the last 4 months. He gave a history of fall followed by the swelling which gradually progressed, without regressing, giving him any pain, tenderness, fever, or unintentional weight loss. There was no history of discharge. Local physicians' medicines didn't help him.

He had a history of appendicectomy 2 years ago, and a treated fracture 10 years ago. All of his 6 brothers and 1 sister were healthy as were his parents.

Examination revealed a healthy looking young boy sitting comfortably, well oriented in time, place and person, having normal vital signs, mild pallor, and a swollen left jaw measuring 6 by 4 sq.cm, non adherent to skin, immobile, without any well defined boundary, with normal skin over it; draining lymph nodes were not palpable. Systemic examination was unremarkable.


His complete blood cell (CBC) parametres showed:

 Table 1: CBC parametres

TLC       11000/uL

TRBC    4.28 (10^6)/uL

Hb          12.4 g/dL

MCV       84 fL

MCH      29 pg

MCHC   34 g/dL

PLT        37000/uL

RDW-SD 45 fL

ANC       7000/uL

ALC        2400/uL

AMC       1130/uL

AEC        1000/uL

ABC        20/uL


  
 
His ESR was 22 mm fall in the first hour. His serology for renal and liver function tests was normal. S. Uric acid was in upper normal range i.e. 410 umol/L (200 - 416). 

An x-ray skull was advised:

 

His X-ray skull was reported as follows: A lytic lesion with multiple septations noted along with half with medial displacement of teeth. No normal bone seen in involved region. Strongly suggestive of Ameloblastoma.

His X-ray Pelvis and Ultrasonography of abadomen were unremarkable.


A CT-Scan was advised which showed:

     

'Aggressive mass left hemimandible of odontogenic/non-odontogenic origin, with bilateral cervical lymphadenopathy at levels I and II. Biopsy correlation suggested'.


His biopsy of left mandible showed plasmacytoma.


 

A bone marrow examination was requested which showed normal plasma cells in marrow:

His urinary Bence-Jones proteins were absent, and protein electrophoresis revealed:


He was diagnosed as having Solitary Plasmacytoma of Bone. His radiotherapy was started which relieved his jaw swelling a lot.



 

 

DISCUSSION



Plasma cell disorders include Plasma cell myeloma (=Multiple Myeloma), Solitary Plasmacytoma of bone, Extramedullary Plasmacytoma, Monoclonal Gammopathy of Undetermined Significance (MGUS), Asymptomatic/Smouldering myeloma, Plasma cell leukaemia, Amyloidosis, Osteosclerotic myeloma (=POEMS syndrome). 

Solitary Plasmacytoma is an uncommon disorder. The criteria for diagnosis includes solitary lytic bone lesion on skeletal survey, histologic confirmation of the lesion, and bone marrow plasmacytosis of <10 %.  Monoclonal protein if detected is cleared after therapy. It usually affects vertebral column. In our patient the site was jaw which is rare.

In patients with low levels of paraprotein, immunofixation should be done to detect presence of small amounts which hide in the gamma globulin spike on protein electrophoresis. In our patient, it could not be done due to non-availability of the patient.