76 year old man with P vera and shortness of breath

1
76 year old man with P vera and shortness of
breath

• Anh Innes, M.D.
• December 10, 2002

2
History

• 76 yo man with polycythemia vera and ulcerative
  colitis presents with LLQ abdominal pain and SOB
• DOE with flights of stairs x 1-2 weeks no
  recent travels, surgeries, or injuries
• Abdo pain worse with eating no changes of
  BM/nausea/vomiting

3
History, cont.

• PMH
• HTN
• UC
• s/p partial colectomy
• P vera
• s/p phlebotomy

• Social
• 3-5 drinks/week
• ? tobacco or illicit drugs
• Retired engineering techician
• married

4
Data

• WBC 25.3, HCT 55.3, ptlts 465
• Other labs WNL

5
(No Transcript)
6
(No Transcript)
7
Data

• CT abdomen
• L ventral hernia
• RLL subsegmental PE
• Small L pleural effusion with LLL air space
  opacity

• VQ scan High-Prob
• R mid lung-field wedge-shaped perfusion defect
• L lung with wedge-shaped defect in lateral basal
  segment LLL

8

• Pt sent home with enoxaparin and warfarin
• 3 days later, presented with increased SOB and
  presyncope

9

• Exam
• mildly tachypneic
• 98.5 85 25-30 150/100 91RA
• Decreased BS at L base, otherwise CTA
• WHSS LLQ with reducible ventral hernia

• Data
• WBC 23.8, HCT 48, ptlts 622
• PT 16.8, INR 1.4
• PTT 36.4
• 7.43/38/55/26/90

10
(No Transcript)
11
(No Transcript)
12
Data, cont.

• Pleural Fluid
• 3.89 x 106 RBC
• 3,800 WBC
• 80 segs, 15 lymphs, 5 eos
• LDH 919
• Glucose 46

• CT of LE
• Multiple r-sided DVT
• R distal superficial femoral v.
• R popliteal v.

13
PE and Hemothorax in P Vera

• Acute management
• IVC filter
• No anticoagulation
• Chest tube
• Prevention of future thrombotic events?

14
Polycythemia Vera

• Chronic myeloproliferate disorder clonal
  proliferation of myeloid cells
• Median age 60, range 20-85
• Dx ? red cell mass, splenomegaly,
  thrombocytosis, neutrophil leukocytosis, absence
  of secondary erythrocytosis
• Untreated patients median survival of 18 months
• Thrombotic complications most common cause of
  death

15
Thrombosis Bleeding in P Vera

• Myeloproliferative diseases hemostatic
  imbalance ? thrombosis and hemorrhage
• Hemorrhage mucocutaneous, GI tract
• Soft-tissue and muscular bleed uncommon
• Thrombosis arterial gt venous
• Pathogenesis platelet abnormalities,
  dysfunction of leukocytes and endothelium

16
Thrombosis Bleeding in P Vera

• Clinical variables
• Age gt60-70
• More severe thrombi ? influence on hemorrhage
• History of prior thrombi
• Hematocrit Hct 60 60 incidence per year
• White cell count interactions with platelets
  and vessel walls
• Platelet count no data proving correlation with
  thrombi and hemorrhage

17
Causes of death in 192 pts with P Vera
Pearson et al. Hematology 2000 (1) 51-68
18
Treatment of P Vera

• Phlebotomy
• 32P
• Hydroxyurea
• Interferon-?
• Anagrelide
• Aspirin

• Goal Hct lt45
• Age gt60, history of thrombosis phlebotomy
  supplemental therapy

19
P Vera, Hydroxyurea, and Thrombosis
20
P vera, Hydroxyurea, and Thrombosis

• Thrombotic complications major cause of death
• The ideal therapy hematologic control,
  reduction in thrombotic events and myelofibrosis,
  no increase in leukemogenesis/carcinogenesis
• Hydroxyurea and thrombosis ? incidence compared
  to phlebotomy and myelosuppressive/alkylating
  agents
• Leukemogenesis and reduction of myelofibrosis
  unknown

21
References

• Donovan PB, Kaplan ME, Goldberg JD, et al.
  Treatment of polycythemia vera with hydroxyurea.
  Am Jrnl of Hematology (1984) 17329-334.
• Landolfi R, Rocca B, Patrono C. Bleeding and
  thrombosis in myeloproliferative disorders
  mechanisms and treatment. Crit Rev in Onc/Hemat
  20 (1995)203-222.
• Fruchtman SM, Mack K, Kaplan ME. From efficacy
  to safety PVSG Report on Hydroxyurea in
  patients with P Vera. Sem in Hemat, (1997)
  34(1)17-23.

• 4. Najean Y, Rain JD. Treatment of P Vera the
  use of hydroxyurea and pipobroman in 292 patients
  under the age of 65 years. Blood (1997) 90(9)
  3370-3377.
• 5. Lofbenberg E, Wahlin A. Management of p vera,
  essential thrombocythemia, and myelofibrosis with
  hydroxyurea. Eur J Haematol (1988) 41375-381.
• 6. Pearson et al. A p vera update. Hematology
  (2000) 151-68
• 7. Tatarsky I, Sharon R. Management of p vera
  with hydroxyurea. Sem in Hemat (1997)
  34(1)24-28.