Thrombotic Thrombocytopenic Purpura Finds Its Way to the Heart
Mamta Parikh, MD and Tim Grennan, MD
University of California, Davis Medical Center; Sacramento, CA
Kaiser Permanente Northern Sacramento; Sacramento, CA
Learning objectives
- Understand the clinical presentation of TTP
- Be aware of reported cardiopulmonary
complications associated with thrombotic
microangiopathies and their mechanisms
Introduction:
- Thrombotic Thrombocytopenic Purpura (TTP) is
characterized classically by neurologic changes, fever,
renal failure, thrombocytopenia, and microangiopathic
hemolytic anemia (MAHA)3,4.
- Only thrombocytopenia and MAHA are required for
diagnosis in the correct clinical context.
- It is rarely accompanied by cardiopulmonary
complications.
Table 1:
9.4
73
16.5
26.8
Image #1:
on admission
Case Information:
A 29-year old woman with a history of chronic abdominal
pain was brought to the Emergency Department. The
patient had a history of one week of diffuse abdominal
pain, diarrhea, progressing to vomiting and then to
lethargy and altered mental status.
On presentation:
- Physical exam was notable only for dilated pupils and
general confusion in response to questions
- At the time of admission, significant laboratory results
are as reported in Table 1.
- CXR at the time is shown in Image #1.
- A peripheral blood smear showed >10%
schistocytes/hpf
Hospital Course:
- Treated with plasma exchange, steroids, hemodialysis,
and intermittent blood transfusions for TTP
- On HD#7, she was found to be hypoxic and in acute
respiratory distress and a CXR at the time (Image #2)
was concerning for bilateral pulmonary edema
- Admitted to ICU, required NPPV
- BNP at the time was 4013.
- Transthoracic echocardiogram:
- Ejection fraction of 40%
- No focal akinesis
- PA systolic pressure of 48 mmg Hg
- She was treated with both continued hemodialysis and
furosemide, afterload reduction with hydralazine
- By HD #9, pulmonary edema had improved
considerably, no longer had respiratory distress.
136 92
4.2 29
Image #2:
HD#7
84
154
8.25
LDH= 7146
Haptoglobin <7
Lactate= 1.3
CK= 98
UA: E Coli UTI
Discussion:
-The pathophysiology of TTP is thought to be due to the persistence of
ultralarge vWF multimers which cause platelet aggregation and
thrombi3,4.
-In autopsies, vWF-rich hyaline thrombi are found in arterioles and
capillaries of various organs, including the heart, kidney, and brain5.
-Prior reports of cardiopulmonary complications:
- Non-cardiogenic pulmonary edema: In a review of TTP cases
from 1981-1998, 7 out of 56 cases of TTP were found to have
ARDS, and in these cases, when plasma exchange was
initiated, there was improvement of ARDS. The mechanism for
ARDS in these cases was postulated to be microthrombi in
pulmonary alveolar capillaries leading to hypoxemia and
pulmonary edema1.
- Mixed cardiogenic and non-cardiogenic pulmonary edema:
Pathologic examination revealed hyaline thrombi deposited in
the heart2.
- Takotsubo’s cardiomyopathy: A case has been reported of a
patient who developed acute cardiomyopathy with TTP,
presumably from a stress response6.
In this case:
-Patient met 4/5 diagnostic criteria for TTP upon admission, lacking
only fever.
-Respiratory distress differential included cardiogenic pulmonary
edema and TRALI (given frequent transfusions). However, she
developed LV dysfunction, and pulmonary edema resolved quickly with
appropriate treatment of heart failure, suggesting that respiratory
distress was more likely cardiogenic.
Clinical importance:
While respiratory dysfunction infrequently accompanies thrombotic
microangiopathies, respiratory status should be monitored vigilantly as
these patients are susceptible to both cardiogenic and non-cardiogenic
pulmonary edema.
References:
1
R. C. Bone, J. E. Henry, J. Petterson, and M. Amare, 'Respiratory Dysfunction in
Thrombotic Thrombocytopenic Purpura', Am J Med, 65 (1978), 262-70.
2
J. C. Chang, and E. S. Aly, 'Acute Respiratory Distress Syndrome as a Major
Clinical Manifestation of Thrombotic Thrombocytopenic Purpura', Am J Med Sci, 321
(2001), 124-8.
3
J. N. George, 'Clinical Practice. Thrombotic Thrombocytopenic Purpura', N Engl J
Med, 354 (2006), 1927-35.
4
J. E. Kiss, 'Thrombotic Thrombocytopenic Purpura: Recognition and
Management', Int J Hematol, 91 (2010), 36-45.
5
H. M. Tsai, 'Pathophysiology of Thrombotic Thrombocytopenic Purpura', Int J
Hematol, 91 (2010), 1-19.
6
H. Zhan, H. Zheng, A. R. Moliterno, and M. B. Streiff, 'Acute Cardiomyopathy in a
Patient with Thrombotic Thrombocytopenic Purpura', Am J Med, 123 (2010), e3-4.