Department of Medicine Grand Rounds Clinical Vignette

1
Department of Medicine Grand Rounds Clinical
Vignette

• Sameer Dhalla, M.D.
• November 15th, 2006

2
Chief Complaint

• 26-year-old South-Asian male who presents with 1
  month of refractory left groin and left leg pain
  along with one day of impaired vision

3
HPI

• Patient was in his usual state of good health
  until 1 month prior to presentation, when he
  developed progressive pain in his left groin,
  7/10 severity, with radiation to his thigh. In
  addition, he noted patches of numbness along the
  anterior lateral thigh and painful hyperesthesia
  of the anterior medial thigh.

4
HPI

• Over the course of the month, he was evaluated
  in the ER twice, given first NSAIDs and then
  corticosteroids, both which failed to palliate
  his symptoms.
• On the morning of presentation, he awoke with
  difficulty focusing vision in his left eye. He
  presented to the ER and was admitted for further
  evaluation

5
Physical Exam

• General A thin, well developed male, appearing
  stated age. Uncomfortable and apprehensive, but
  in no acute distress.
• VS T 98.3 HR 75 BP 106/65 RR 16 Sat
  100 RA
• HEENT Mild conjuctival pallor. Decreased left
  eye visual acuity. Left retinal hemorrhage on
  fundoscopy.
• ABD Spleen tip palpable in right hemiabdomen 2
  cm beyond umbilicus
• Neuro Left thigh numbness to light touch and
  pinprick along lateral anterior surface.
  Increased pain to light touch along medial thigh.
  

6
Labs
Na 141 K 4.5 Cl
105 CO2 25 BUN 15 Cr
0.8 Glucose 80
WBC 320,000 Hgb 8.2 HCT
25.3 PLT 251 MCV 83

PMN 79 Lymph 16 Blasts
3 Promyelocyte 2
7
Radiology

• Head CT Left retinal hemorrhage
• CT lumbar spine Bilateral 4x6 cm fluid
  collection, measuring 39-44 HU, located anterior
  to the iliacus muscle. DDx Hematoma vs.
  Leukemia. No cord compression

8
Preliminary Diagnosis

• Chronic Myelogenous Leukemia

9
Therapeutic Course

• Upon admission, transfused with packed red blood
  cells. Given 3 days of Hydroxyurea
  pharmacotherapy. On HD3, started Imatinib
  Mesylate (Gleevec) 400 mg PO daily.

10
Bone Marrow Biopsy Hypercellular marrow, no
blasts seen, increased ME ratio Cytogenetics
922 translocation (the Philadelphia chromosome)
seen in 20/20 metaphases
11
Therapeutic Course

• Patient was hospitalized for 1 week, over which
  time his symptoms and blood counts demonstrated a
  response to Gleevec. Discharged on Gleevec 400 mg
  PO daily.
• Karotype performed following 3 months of therapy
  demonstrated 3/17 metaphases with 922
  translocations. FISH demonstrated 1 cell (0.4)
  with a double Philadelphia chromosome (no prior
  FISH performed). Gleevec increased to 800 mg for
  concern of accelerating disease. 1 month later,
  complete remission identified on bone marrow
  biopsy. Patient was now asymptomatic. Fluid
  masses no longer present on repeat CT of lumbar
  spine

12
Therapeutic Course

• Patient has now been taking Imatinib for over 24
  months with no clinical or laboratory evidence of
  CML recurrence. Although 3 HLA 9/10 unrelated
  C-locus mismatches exist, stem cell
  transplantation is currently being deferred.
• Pt has reported only minor side-effects,
  including lower extremity vitiligo, intermittent
  hypophosphatemia.

13
Final Diagnosis

• Chronic Myelogenous Leukemia in Accelerated Phase
  with Extramedullary Involvement