Radiology Case Conference

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Radiology Case Conference

• July 2, 2002
• Paresh Patel, MD

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History (2/22/02)

• 43 yr old white female with no significant PMH
  except hyertension
• C/C cough-3 wks
• SOB-3 wks

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HPI

• Cough-progressively getting worse associated
  with yellow sputum for 3wks
• No hemoptysis
• SOB present for 3 wks getting worse
• No chest pain, fever, headache, nasal symptoms
• Wt loss 30 lbs-intentional has been trying to
  loose wt for some time.

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HPI contd

• H/o exposure to co-worker with TB
• No pets, travel history
• Evaluated by private MD earlier in Feb 2002 who
  detected CXR abnormalities, low oxygen saturation
  and referred for further workup.

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PMH

• Hypertension
• Obesity
• Breast biopsy in 1991-negative for malignancy

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• PSHx Fracture radius in 1997
• Allergy PCN, tetracycline
• Medications Diovan (Valsartan) 80 mg qd
• Birth control pills

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• Personal/Social Hx lifelong non-smoker, no h/o
  ETOH/drug use.
• Married, works as a supervisor
• Family Hx Mother died of Br cancer at age 44
  yrs.
• ROS Negative
• PPD status unknown

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Examination

• Vitals
• Temp 36.5 oC Pulse rate 74/min RR 20/min
  BP 108/62 mm Hg SpO2 94 on 4 L NC
• Wt 250 lbs
• HEENT normal
• Neck no LNE
• Breast no lumps
• Chest bibasal crackles, no wheezing/ronchi/rub

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Examination contd.

• CVS Normal S1 S2. No murmurs
• Abdomen Liver,spleen-not enlarged, no masses
• CNS no neuro deficits
• Skin no rash
• Ext no edema

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Laboratory

• CBC (2/22/02)
• WBC8.3 Hb12.9 Hct38.5Plt248
• Chemistry (2/22/02)
• Na141, K4.5, Cl107, CO225, BUN11, Cr1.1,
  glucose136, Ca8.8
• PT/PTT/INR12.2/30.6/0.93
• ABG (2/12/02) 7.46/34/51/89 RA
• (2/22/02) 7.40/39/80/95 4 L NC

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Chest X-ray
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Picture 1 (2/22/02)
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Picture 2 (2/22/02)
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Picture 3 (3/27/02)
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Picture 4 (3/27/02)
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Picture 5 (5/23/02)
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Picture 6(5/23/02)
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Picture 7 (6/23/02)
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Picture 8 (6/23/02)
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CT Scan
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Picture 9 ((2/13/02)
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Picture 10 (2/13/02)
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Picture 11 (2/13/02)
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Picture 12 (2/13/02)
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Picture 14
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Picture 13
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Differential Diagnosis

• Metastatic disease
• Sarcoidosis
• TB/Histo/crypto/nocardia/coccidiomycosis
• Rheumatoid lung
• Wegners granulomatosis
• Multiple pulmonary abscesses

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More labs

• RF lt20
• ANA negative
• C-ANCAnegative
• P-ANCAnegative

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Give me the tissue!
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Bronchoscopy (2/15/02)

• No endobronchial lesion, yellowish secretions
• Cytology (Bronchial washings brushings) neg
• Transbronchial Biopsy (RML, RLL)
• No active inflammation, few intra-alveolar
  macrophages, interstitium showed few granulocytes
  lymphocytes, no granuloma seen, no evidence of
  malignancy
• Special stains negative
• Post-bronch sputum cytology negative

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Open lung biopsy (2/22/02)

• Gross (RUL,RML,RLL wedge)
• Ill-defined fleshy lesions 1.0 cm to 1.5 cm

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Picture 15
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Picture 19
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Picture 16
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Picture 18
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Picture 20
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Picture 21
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Picture 22
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Microscopic

• Nodular infiltrate of lymphocytes histiocytes.
• No necrosis
• Lymphocytes do not show significant cytological
  atypia
• No well formed granulomas.
• T-cell preponderance with scattered B-cells
• EBV stains negative
• No clonal rearrangements by PCR for Ig heavy
  chain gene rearrangement T cell receptor gene
  rearrangement
• Flow Cytometry no unique cell populations

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Final diagnosis

• Atypical lymphohistiocytic infiltrate c/w
  Lymphamatoid Granulomatosis-Grade I

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Hospital Course F/U

• Uneventful hospital course.
• CT removed on 2/27/02
• d/ced on 2/28/02

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Lymphamatiod Granulomatosis

• First described by Liebow and colleagues in 1972
• Angiocentric lymphoproliferative disease
• Unknown etiology
• Skin and pulmonary parenchymal involvement
• Presents in adults as cough,dyspnea, and
  occasionally sputum production, fever and malaise
• CNS involvement in one-fourth of patients,
  characterized by focal deficits in asymmetric
  distribution, sometime with seizures.
• Dermatologic involvement in ½ of patients, with
  patchy erythematous macular or papular areas,
  usually small and non-confluent. Lesions mostly
  occur on the extremities and may ulcerate,
  subcutaneous nodules also occur.

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• Pulmonary involvement-invariably present as
  parenchymal masses, usually in the periphery and
  lower lobes.
• Extensive destruction of lung parenchyma
  sufficient to cause respiratory insufficiency has
  been reported as well as occasional occurrence of
  massive hemoptysis or severe cavitory disease.
• Airway infiltration may also occur, usually in
  bronchioles, but lobar obstruction by
  endobronchial destructive lesions has been
  reported.

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• Kidney involvement rare unlike Wegners
  granulomatosis. focal necrotic and proliferative
  lesions are seen without glomerulonephritis.
• Hepatic involvement, although unusual, may carry
  a worse prognosis.
• Lymph node and splenic involvement-rare, does not
  affect prognosis.
• Rarely involves the nasopharynx and upper
  airways.

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Diagnosis

• histologically-destructive, inflammatory
  granulomatous angiitis. In contrast to Wegners
  granulomatosis, the lymphoid infiltrate is
  composed of atypical immature cells with
  abundant mitoses a paucity of polymorphonuclear
  or eosinophilic leukocytes. These infiltrates
  have been shown to be B cells, usually with EBV
  RNA within them surrounding reactive T cells.
• X ray findings-not unique. Multiple bilateral
  pulmonary nodules, cavitations, atelectasis or
  lobar destruction, large mass like lesion
  pneumonthorax may be seen. Pulmonary lesions may
  wax and wane. Hilar LNE is rare so are normal
  chest radiographs

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Pathogenesis

• Unknown
• Although EBV is frequently found in the B cells
  in the granulomas, no investigator has
  demonstrated a causative relationship.
• Both monoclonal T-cell and B-Cell proliferation
  have been identified in this disease.

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Treatment

• Significant patients have benign course and may
  not require treatment
• Symptomatic pts treated with steroids and
  antineoplastic drugs, predominantly low dose
  Cyclophosphamide
• Usually responsive, however recurrs and
  development of either refractory disease or
  high-grade lymphoma is common.
• Radiotherapy works for localized lesions.
• Prognosis is poor with half or more patients
  succumbing to the disease within 5 yrs.
• The disease terminates in aggressive lymphoma in
  15 to 25 of patients.