Case Discussion Burkitt ’ s Lymphoma with Central Nervous System Relapse 指導醫師 : VS 蘇裕傑醫師 實習醫師 : Intern 傅斯誠醫師 2005/11/05.

Case DiscussionCase DiscussionBurkitt’s Lymphoma with Burkitt’s Lymphoma with Central Nervous System Relapse Central Nervous System Relapse

指導醫師 : VS 蘇裕傑醫師實習醫師 : Intern 傅斯誠醫師

2005/11/05

Patient Data

盧 先生19 year-old male

ID: I100150062

Admission date: 2005/10/12

Chief Complaint:

Patient Data

盧 先生19 year-old male

ID: I100150062

Admission date: 2005/10/12

Chief Complaint:Bilateral leg weakness and numbnessBilateral leg weakness and numbness

For 1 dayFor 1 day

Past History

2005/01 Burkitt’s lymphoma

Completed 10 courses of chemotherapy

Present Illness

2005/01Abdominal fullness and poor appetite

Hospitalized at 台南市立醫院Gastric ulcer and ascites

Present Illness

2005/01Abdominal fullness and poor appetite

Hospitalized at 台南市立醫院Gastric ulcer and ascites

Transferred to 嘉義基督教醫院Abdominal Imaging revealed masses

Suspect intra-abdominal lymphoma

Transferred to 台北恩主公醫院CT-guide biopsy for diagnosis

Burkitt’s lymphoma

Liver metastasis

Transferred to 台北恩主公醫院CT-guide biopsy for diagnosis

Burkitt’s lymphoma

Liver metastasis

Transferred to 台大醫院Port-A insertion and Chemotherapy

Transferred to 台北恩主公醫院CT-guide biopsy for diagnosis

Burkitt’s lymphoma

Liver metastasis

Transferred to 台大醫院Port-A insertion and Chemotherapy

Transferred to 大林慈濟醫院Completed 10 courses of chemotherapy

(2005/01/14 ~ 2005/09/23)

2005/01/14 Abdominal CT

2005/01/17

Bone scanNo bony lesions

2005/01/18

Gallium scan

DiagnosisIntra-abdominal origin

Burkitt’s lymphoma

Liver metastases

DiagnosisIntra-abdominal origin

Burkitt’s lymphoma

Liver metastases

Chemotherapy regimen:EPOCH (x2)

High dose MTX + LV + Ara-C

Endoxan + Mesna + Oncovin + Epirubicin (x4)

+ IT Methotrexate and Ara-C

DiagnosisIntra-abdominal origin

Burkitt’s lymphoma

Liver metastases

Chemotherapy regimen:EPOCH (x2)

High dose MTX + LV + Ara-C

Endoxan + Mesna + Oncovin + Epirubicin (x4)

+ IT Methotrexate and Ara-C

Completed on 2005/09/23Completed on 2005/09/23

2005/10/11 15:00

Came to our Emergency Dept.

Chief complaint:General weaknessGeneral weakness

DizzinessDizziness

DyspneaDyspnea

At our ER…

Lab data revealed hypokalemia (K+2.hypokalemia (K+2.8)8)

Given K+ supplement

Allowed discharge

2005/10/12 08:00

Returned to our Emergency Dept.Bilateral lower leg weakness, numbnessBilateral lower leg weakness, numbness

Drooped right faceDrooped right face

DiplopiaDiplopia

Stool IncontinenceStool Incontinence

Social History

No smoking, betel nut, or alcohol use

Lives at home with family

Family HistoryNo family member with tumor history.

No known allergies

Allergy

Physical Examination

Weight: 58kg Height: 178cmVital signs:

TPR: 37.3°C / 98bpm / 20TPR: 37.3°C / 98bpm / 20BP: 127/85 mmHg.BP: 127/85 mmHg.

Skin: normal skin turgor

Head & Skull:Bold, no OP scars

Eyes:Pupils 3.0 / 3.0 Light reflex sluggishConjunctiva pink

Physical Examination

ENT & Mouth:Hearing normal, oral mucosa intact

Neck:No jugular vein engorgement, no carotid bruits

Neck movement normal, no palpable lymph nodes

Thyroid gland impalpable

Chest & Lungs:Breathing sounds regular, bilateral expansion symmetric

Heart: Heart sounds regular, no murmurs.

Physical Examination

Abdomen:Flat, soft, no tenderness

Liver and spleen impalpable.

Extremities:Movement of upper extremities normal

Movement of lower extremities ok, but weakMovement of lower extremities ok, but weak

Back & Spine:No kocking pain over C-V angles

Neurological Examination

Level of consciousness : clear, alert Mental status normal

JudgementOrientationMemoryAbstract thinkingCalculation

SpeechContent logical, comprehensibleArticulation slightly unclearArticulation slightly unclear

Neurological Examination

Cranial nerves :CN I: no loss of smellCN II:

Pupils isocoric 3.0 / 3.0, light reflex sluggish Visual field normalVisual acuity well

CN III, IV, VI: Left eye lateral movement impairedLeft eye lateral movement impaired

CN V: Normal muscle power of masseterNo numbness over faceCorneal reflex normal

Neurological Examination

CN VII: Right facial expression impairedRight facial expression impaired

Peripheral type Bell’s facial palsyPeripheral type Bell’s facial palsy

CN VIII: hearing normal

CN IX, X: Phonation normal

Swallowing normal

No deviation of uvula

CN XI: Normal muscle power of S.C.M & trapezious m.

CN XII: Leftward deviation of tongueLeftward deviation of tongue

Neurological Examination

Motor system:Bilateral lower extremity weaknessBilateral lower extremity weaknessStool Incontinence (+)Stool Incontinence (+)

Neurological Examination

Motor system:Bilateral lower extremity weaknessBilateral lower extremity weaknessStool Incontinence (+)Stool Incontinence (+)

Sensory system :Decreased sensation over right lateral thighDecreased sensation over right lateral thigh

Neurological Examination

Motor system:Bilateral lower extremity weaknessBilateral lower extremity weaknessStool Incontinence (+)Stool Incontinence (+)

Sensory system :Decreased sensation over right lateral thighDecreased sensation over right lateral thigh

Cerebellar function:F-to-N : intactRAM : intactTruncal ataxia : nil

Neurological Examination

Motor system:Bilateral lower extremity weaknessBilateral lower extremity weaknessStool Incontinence (+)Stool Incontinence (+)

Sensory system :Decreased sensation over right lateral thighDecreased sensation over right lateral thigh

Cerebellar function:F-to-N : intactRAM : intactTruncal ataxia : nil

Deep tendon reflexDiffuse decrease of DTR

Summary ofNeurological Findings1) Left eye deviation

2) Right Bell’s palsy

3) Tongue deviation

4) Right thigh numbness

5) Bil. lower extremity weakness

6) Stool incontinence

7) Diffuse decrease of DTR

Summary ofNeurological Findings1) Left eye deviation (CNIII, VI)

2) Right Bell’s palsy (CNVII peripheral)

3) Tongue deviation (CN XII)

4) Right thigh numbness (L1)

5) Bil. lower extremity weakness (PT)

6) Stool incontinence (Spine)

7) Diffuse decrease of DTR (K+)

Lab Data

Upon Admission…

<CBC>

<CBC>

2005/10/11

PA CXR

Problem List

Burkitt’s lymphoma with CNS replapse

Hypokalemia, Hyponatremia

Treatment Plan

Burkitt’s lymphoma with CNS replapse Bone marrow aspirationBone marrow aspiration

CSF studyCSF study

Intra-thecal chemotherapyIntra-thecal chemotherapy

CNS RadiotherapyCNS Radiotherapy

Hypokalemia, HyponatremiaK+, Na+ supplementK+, Na+ supplement

Bone Marrow Aspiration 10/12

Large lymphocytes

>Blue cytoplasm

>Vacuoles

Bone Marrow Aspiration 10/12

Large lymphocytes

>Blue cytoplasm

>Vacuoles

RELAPSE!RELAPSE!

2005/10/12 Lumbar puncture

2005/10/12 Lumbar puncture

Cytology:Burkitt’s lymphoma with CNS involvementMassive tumor cells with large nucleus, scanty cytoplasm

Intrathecal methotrexate

2005/10/12 Lumbar puncture

2005/10/12 Lumbar puncture

Cytology:Burkitt’s lymphoma with CNS involvementMassive tumor cellsSome cell necrosis

Intrathecal methotrexate

2005/10/17 Lumbar puncture

2005/10/17 Lumbar puncture

Cytology:Burkitt’s lymphoma with CNS involvementSome tumor cellsCell necrosis

Follow-up Conditions

10/16Spontaneous stool passageAble to stand, walk slowly

Follow-up Conditions

10/16Spontaneous stool passageAble to stand, walk slowly

10/17Left eye lateral movement (+)Walking improvedSwallowing improvedDTR (+)

Questions?Questions?

DiscussionBurkitt’s Lymphoma with Burkitt’s Lymphoma with

Central Nervous System RelapseCentral Nervous System Relapse

DiscussionBurkitt’s Lymphoma with Burkitt’s Lymphoma with

Central Nervous System RelapseCentral Nervous System Relapse

IT HAPPENS!IT HAPPENS!

Natural Course

Burkitt's Lymphoma CNS involvement: 20~30%

Presentation?Risk factors?Benefit?Prognosis?CNS prophylaxis regimen?

Presentation

The commonest featuresHeadache

Cranial nerve palsies

Spinal cord compression

Altered mental state and affect

Central Nervous System LymphomaAndrew Lister, Lauren E. Abrey, and John T. Sandlund, Hematology 2002

Risk Factors

Risk Factors

1980~1996Norwegian Radium Hospital 2514 Non-Hodgkin Lymphoma patientsWithout CNS presentationRetrospective analysis

Risk Factors

Non-Hodgkin’s Lymphoma

Age > 60 years old

LDH > 450 U/L

Albumin < 35 g/L

Retroperitoneal gland involvement

Extranodal sites >1

Burkitt’s type is a risk factor! (24%)

Useful for High-grade NHL

Benefit of Prophylaxis

CNS involvement in Burkitt’s (at 5 years)Overall 24%Without prophylaxis 78%With prophylaxis 19%

Central Nervous System involvement following diagnosis of non-Hodgkin’s Central Nervous System involvement following diagnosis of non-Hodgkin’s lymphoma: a risk model lymphoma: a risk model A. Hollender et alA. Hollender et al. Annals of Oncology 2002. Annals of Oncology 2002

Prognosis

CNS involvement to deathMedian survival

Primary progression 2.4 Months

Relapse 2.2 Months

Regimen

Regimen

2004 Feb.~Apr. (159 UK Medical Centers)293 questionnaires

158 Received65 Followed by telephone7 0 Did not care for NHL patients

96%96%

Back to our patient…

Presentation

Risk Factors

Regimen

Prognosis

Back to our patient…

PresentationTypical relapse

Risk FactorsBurkitt’s type high risk

RegimenMTX based (+Ara-C)

PrognosisPoor

Back to our patient…

PresentationTypical relapse

Risk FactorsBurkitt’s type high risk

RegimenMTX based (+Ara-C)

PrognosisPoor

Comments?

Discussion comments

1) If patient turned out to have normal CSF study, what is our next step?

Cancinomatosis of meninges can also be diagnosed through MRI image studies.

2) The journals involved in this discussion did not help with patient’s future management. What are some other topics of consideration in the benefit of our patient?

The discussion included here focused mainly on statistical analysis of the course of Burkitt’s lymphoma. Of course, newer studies on autologous stem cell transplant for cure are also being carried out. This topic should also be included here.