1
Case Presentation

• Dhyia Al Qutshan, M.D
• Moderators Riyad Sayd, MD
• Ahmed Malouh, MD

2
History of present illness

• K.H is 6 year old male patient known case of
  C.R.F secondary to reflux nephropathy S/P renal
  transplant done Egypt from non-related living
  donor (NRLD) 8 months prior to admission???
• Presented with a mass on the right side of the
  neck that increased in size over the last two
  months.

3

• History of runny nose ,cough and right A.O.M
  that was treated with antibiotics prior to the
  mass appearance.
• The neck mass was thought to be secondary to the
  U.R.I/A.O.M.
• The neck mass has failed to decrease in size and
  started to become larger with time.

4
ROS

• History of decrease appetite but no history of
  weight loss
• No history of chest pain, SOB, tremors,
  svisual/speech disturbances.
• No history of vomiting, diarrhea, abdominal pain
  or distention.
• No history of frequency, urgency, dysuria
  hematuria.
• No history of joint swelling ,redness, pain
  ,limitation of movements.
• No history of skin skin rash.
• No history of change in level of consciousness ,
  sensual or speech disturbances.

5
Cont..

• Immunization UTD
• Development attends daycare with no concerns.
• Social history lives with both parents and
  siblings, insured , no concerns
• Family history no history of kidney diseases,
  HTN, DM , malignancies.

6

• Tru-cut biopsy of cervical mass was
  done(5/1/2009) in one of the West Bank Hospitals
  Necrotic tissue (repeat or excisional biopsy
  is recommended)

7
Medications

• Prograf GT 3 mg bid
• Diltizam GT 30 mg Qd
• Ferrous sulfate 125 mg
• Predinosolon 10 mg qd
• CaCO3 600 mg
• Bactrim 5 ml qd
• ASA 100 mg qd
• Calcipt 500mg bid
• No adverse interactions

8
Physical Examination

• V/S T37.0 (O) RR22 BP 130/70
• Wt 17.0 kg 5th HT 108 cm 5th
• General patient looked well, not in acute
  distress.
• HEENT PERRL, right side swelling at the base of
  the neck 3 4 cm firm-hard, non tender, slightly
  mobile ,no discoloration, Single LN palpable
  (anterio cervical )12 non tender ,mobile,firm.
• No tonsilar enlargement or redness, TM N/L B/L

9

• Chest CTA B/L No wheezing No crackles
• Heart NL S1 S2 no Murmur
• Abdomen No distention ,Soft ,lax ,BS active ,no
  tenderness, no HSM
• Ext No swelling , discoloration.
• Neurology Cranial nerve 2-12 intact Power 5/5
  ULs LLs Reflex 2 Knee,ankle biceps. Normal tone
  and gait
• Cerebellar signs ve,

10
Labs upon Admission

• WBC 7.9 Neuts 71 Lymph 21 Mono 8
• HgB 7.5 PCV 23.2 MCV 76 MCH 25
• PLT 619
• Blood film RBC are decreased in number and are
  borderline hypochromic microcytic.WBC are normal
  in number , no blasts.PLT normal morphology.

11

• BUN 136 CRT 2.3 K 4.9 Ca 9.6 PO4.8 LDH
  317 U.A 10.5 TBIL 0.3 DBIL 0.08 ALK 57 SGOT 18
  SGPT 11 T.PROT 7.2 ALB 4.8 ESR 85

12

• PT 13.2 PTT 22.2 INR 1.02 Prograf 5.8
• HBsAg ve, HIV ve, HCV ve
• UA SG 1010 Ph 6.0 protein trace
• glucose 1 fine granular cast , WBC 8, RBC 2
  nitrate -ve

13

• CXR increased bronchovasscula marklings
  bilateral No consolidation.No mediastinal masses.

14
Bone marrow biopsy and aspirate bilateral

• Reactive hypercellular bone marrow
• No evidence of bone marrow infiltrate or fibrosis.

15
Incisional biopsy

• Origin Right neck mass
• Microscopic Exam extensive coagulopathy necrosis
  of lymph node tissue .Few surviving large
  abnormal anaplastic lymphocytes having large
  irregular nuclei and prominent nucleoli
• Immunohistochemistry the lymphoid cells strongle
  positive for CD20
• DiagnosisMonomorphic post-transplant
  lymphproliferative disease(large B-cell lymphoma)

16
Right transplant kidney biopsy

• Significant tubular changes,some are acute with
  regenerative changes ,others show infiltrate by
  lymphocyte .Mild interstial lymphocytic
  infiltrate is noticed with focal interstial
  fibrosis.No lymphomatous infiltrate is seen.
• Immunoflourescence Negative for IgA,IgG,IgM,C3,C4
  and fibrinogen
• Immunohistochemistry C4d stain is negative
• Diagnosis Acute Tubulo-interstitial rejection.

17
C.T Head and Neck

• 45 heterogeneous mass in the right side of the
  neck antero-medial to the SCM.No evidence of
  other gross cervical LN enlargement
• Hypophrynx and Larynx appear normal.
• DDx L.N( milgnant ie metz, lymphoma or
  infilmmatory ) Vs infected 2nd branchial cyst

18
Chest CT

• Clear lung field, Mediastimum and hila appear
  normal

19

• Well defined mass about 4 cm with enhancing
  capsule seen adjacent to the upper pole of the
  left kidney indenting the spleen (origin splenic
  Vs renal)
• round hypodense lesion 2 cm in the lower aspect
  of the spleen(lymphomatous infiltrate)
• Abdominal CT Liver appear normal in size and
  homogenous.
• Native Kidneys appear small and atrophic

20
Diagnosis

• Monomorphic post-transplant lymphproliferative
  disease PTLD (large B-cell lymphoma)
• Strongly positive for EBV(LMP-1)

21

• Ks was given solumderol (pulse therapy)170 mg iv
  daily for three days
• Ratixumab,cyclophosphamide prednisolone
• Labs Bun 86, crt 0.9 na 144 k 4.3 ca 9.5 po4 3.7
• d/c medications prograf 2mf bid dilatrend 6.25 mg
  bid feso4 5 ml, ca carbonate 600 mg bid asa 100
  mg dilzem 30 mg qd nexium 20 mg daily,
  prednisolone 10 mg
• d/c calicept