IT WAS SENT TO MY BLOG.....HOW TO KNOW YOUR CHILD HAS Burkitt Lymphoma

 

•       First described in 1910 by Dennis Burkitt, a British surgeon working in Uganda who called it jaw sarcoma

•       Epidemiology

–      Most common childhood malign in the tropics

–      Incidence is 1:10,000

–      M/F is 2:1

–      Age range is 4-9years, mean of 7yrs

–      2 types-endemic type seen in the tropics (10-15o north and south of the equator, annual rainfall >50cm all year round, temp not less than 26.6oC

–      Sporadic type seen South America and Papua New Guinea

CLINICAL FEATURES

•       Is the fastest growing tumor known to man with a doubling time of 24hrs

•       Presents as facial tumor and or Abdominal mass

•       Facial mass

•       Jaw swelling which is painless associated with loosening of the teeth and mal-alignment of the teeth (dental anarchy) with intra oral extension associated with soft tissue swelling

•       Proptosis occurs from orbital extension of  maxillary tumor

Abdominal tumor

•       Progressive abdominal swelling associated with pain. Mass is hard and craggy. May involve paired organs like the ovaries or kidneys. Also associated with ascites

CNS involvement

•       Is due to CNS metastasis resulting in cranial nerve deficits and signs  or paraplegia due to extradural cord compression by abd masses

•       Less common sites of involvement  are the bones marrow.

Rare it  presents as ALL (L3 subtype) i.e. Burkitt leukemia

INVESTIGATION

                              Biopsy

–      Cytology- burkitt cells are homogenous cells  with round or oval nuclei with 2-5 prominent nucleoli

–      Histology is described as starry sky appearance burkitt cells (sky) interspaced with scattered macrophages (stars)

Radiology

          -jaw- lat oblique view will show  osteolytic bone lesion, dental anarchy, loss of lamina dura

          -myelography in CNS involvement

          -abdominal  UTZ scans

          -CTscan

•       FBC baseline for tx and possible marrow involv

•       E/U : tumor lysis syndrome

•       LDH- useful marker of tumor burden, used to monitor response to tx and determine relapse

TREATMENT

•      COMP (cyclophosphamide, oncovir, methotrexate, prednisolone) with CNS prophylaxis or treatment with IT methotrexate or Ara-C every  2weeks for 6 cycles

•      CHOP – cycloph. Doxo, oncov, predn

•      CHOP+METHOTREXATE

•      Newer drugs- ifosfamide, etoposide, cytarabine,etc

•        

•      Allopurinol-xanthine oxidase inhibitor or recombinant urate oxidase (rasburicase)

•      Hydration 2-3L/m2

•      Alkalinization of urine with NaHCO3 to maintain urine pH at 7-8, prevents precipitation of uric acid

•      Treatment of infections with a/biotic