Open Access

Cryptogenic hepatic insult, failing heart and advancing age: a case report

  • Akansha Agrawal1,
  • Manish Soneja1,
  • Ashish Goel1Email author,
  • H Pati1 and
  • Aparajit B Dey1
Cases Journal20081:408

DOI: 10.1186/1757-1626-1-408

Received: 11 September 2008

Accepted: 19 December 2008

Published: 19 December 2008

Abstract

Background

Weakness and fatigue are accepted as normal accompaniments of aging. Usually, older individuals are not investigated with much enthusiasm but a treatable cause is discernible on several occasions.

Case presentation

We had a 67 year old hypertensive lady with a mitral stenosis, presenting in ischemic or hypertensive heart failure with underlying valvular disease, without pulmonary hypertension in sinus rhythm. She had pancytopenia with severe anemia and raised liver enzymes. Bone marrow examination showed aplastic anemia. She was treated with ATG and improved subsequently to become transfusion free. However, she succumbed to an unrelated sudden cardiac death.

Conclusion

Our patient is unique in her uncommon presentation, complex management issues and a favorable outcome after a long and persevering therapeutic intervention and finally her sudden death.

Case report

In December, 2007 a 67 year old hypertensive lady, with a known rheumatic mitral stenosis, presented with insidious onset, gradually progressive fatigue of one month, which had decompensated acutely. There was no history of peptic ulcer, use of NSAIDs or change in bowel habits. She had no previous blood transfusions or jaundice. She denied smoking or taking alcohol. There was no suggestion of long standing liver or kidney disease, diarrhea or infection. She took amlodipine and atenolol for hypertension. There was no history of intake of any other drugs that could have had a toxic potential.

She was pale, anicteric, normotensive and tachypneic with pulse of 100/minute. Her neck veins were engorged and she had pedal edema. She was afebrile, did not have any clubbing, and had no signs of rheumatic activity or infective endocarditis. She had a loud first heart sound, a normal second heart sound, an opening snap and a mid-diastolic murmur at apex. She had a resonant percussion note, equal air entry, and vesicular breath sounds on both sides. Coarse rales were heard in the infrascapular and infraaxillary areas. She had an enlarged tender, firm liver with sharp margins and a span of 10 cms. No splenomegaly or ascites were noted. Here, we had an old hypertensive lady with a mitral stenosis, presenting in ischemic or hypertensive heart failure with underlying valvular disease, without pulmonary hypertension, rheumatic activity or infective endocarditis in sinus rhythm. An infective pathology causing acute deterioration or a pulmonary embolism was also considered.

Her hemoglobin was 65 g/L, TLC 3.2 × 109/L with absolute neutrophils count of 1.6 × 109/L, platelet count of 43 × 109/L and MCV 110 fL (see figure 1). Macrocytosis, hypochromia, leukopenia and reduced platelets but no abnormal cells were seen. Blood cultures were sterile. She had raised serum bilirubin (2 mg/dL) and liver enzymes (SGPT 877 and SGOT 1179 IU). Serology for hepatitis virus A, B, C, E and HIV was negative. Coombs test and antinuclear antibodies were negative. Serum vitamin B12 level was 896 pg/ml.
Figure 1

Distribution of hemoglobin level, platelet count and total leukocyte counts over time. To represent comparative patterns of platelets, hemoglobin and leukocyte count in the same figure, the platelet count has been represented as actual count * 10-4, leukocyte count has been represented as actual count * 10-3 and the hemoglobin has been represented as its actual value.

Echocardiography showed mild mitral stenosis (MVOA 1.8 sqcm) with normal left ventricular ejection fraction, and no signs of infective endocarditis. Serum and urine electrophoresis did not detect any abnormal bands. Bone marrow biopsy from the iliac crest showed profound hypoplasia with overall cellularity of less than 5%. She had osteoporosis with a bone mineral density of 0.714 g/sqcm. Her liver function tests rapidly returned to normal levels.

She was given immunosuppression with anti-thymocyte globulin at the dose of 40 mg/kg daily for 4 days along with prednisolone at 1 mg/kg tapered over 3 weeks. This was followed by cyclosporine at the dose of 10 mg/kg/day. The patient continued to need regular and frequent blood and component transfusion support for ten more weeks. The profile of her hematological parameters over this period is reflected in table 1. When seen in April, she was doing well and had been transfusion free for three weeks. She subsequently remained free of complications for another month, but then she complained of acute abdominal pain and succumbed before she could be taken to the hospital for medical attention suspected to have had an unrelated sudden cardiac death. A post mortem examination could not be performed.
Table 1

Progression of the hematological parameters of the patient over time

Date

Hemoglobin

Platelet Count

Total Leukocyte Count

30.10.07

9.6

.

7200

11.12.07

6

.

.

12.12.07

5.3

5000

3620

13.12.07

6.9

34000

3400

14.12.07

7.4

40000

2500

15.12.07

6.2

28000

4200

16.12.07

8.5

40000

4000

19.12.07

11.9

.

5500

20.12.07

11.8

10000

3800

21.12.07

10.5

16000

4400

22.12.07

10.8

18000

2900

23.12.07

7.9

17000

4800

24.12.07

9

17000

3200

26.12.07

9.3

26000

2100

27.12.07

7.8

20000

2400

29.12.07

8.3

122000

1600

31.12.07

7.4

88000

1900

14.01.08

8.6

61000

2800

18.01.08

7.8

61000

3100

22.01.08

7.5

24000

4130

24.01.08

9.2

17000

3500

27.01.08

9.4

25000

4400

28.01.08

10.1

15000

5100

31.01.08

8.4

15000

2000

02.01.08

7.7

57000

1900

15.02.08

5.8

13000

7540

17.02.08

8.7

34000

3800

19.02.08

7.7

43000

6130

23.02.08

6.9

18000

5970

26.02.08

6.6

19000

3920

29.02.08

8.9

16000

.

03.01.08

7.4

45000

4200

03.03.08

.

20000

.

14.03.08

11

34000

5000

17.03.08

11.4

40000

8040

04.01.08

6.8

68000

4500

04.02.08

8.7

40000

5500

06.01.08

6.7

13000

1200

07.01.08

6.2

124000

1900

07.03.08

8

19000

8900

08.02.08

7.7

10000

5100

09.01.08

6.4

10000

1800

09.02.08

7.2

6000

3700

10.01.08

6.4

19000

2300

10.03.08

7.3

17000

3670

11.01.08

10.2

19500

4600

11.02.08

8.2

56000

4320

12.03.08

11.6

40000

.

Weakness and fatigue are accepted as normal accompaniments of aging. Usually, older individuals are not investigated with much enthusiasm but a treatable cause is discernible on several occasions. Here, anemia with CHF was evident at presentation. Chronic disease, iron deficiency, vitamin B12 or folate deficiency, gastrointestinal bleeding and myelodysplastic syndrome are commonly identified.[1] Aplastic anemia, remains rare in older persons. Older patients are usually ineligible for allogeneic bone marrow transplantation, owing to absence of a donor, advanced age and frail phenotype. Immunosuppression with cyclosporin and antithymocyte globulin (ATG) is often contemplated but infrequently tried in older individuals.[2, 3] It is known that 50% younger patients respond within 3 months of immunosuppression, and about 75% by 6 months, and become transfusion independent, but some may have a persistently hypoproliferative marrow. In the current case, bone marrow suppression followed a transient cryptogenic hepatitis, likely of a viral etiology. Aplastic anemia has been reported between 3–6 months following cryptogenic hepatitis in younger patients.[4, 5] A more protracted course might be seen in older patients following immunosuppression. Our patient is unique in her uncommon presentation, complex management issues and a favorable outcome after a long and persevering therapeutic intervention and finally her sudden death.

Consent

Consent could not be taken from the patient before publication because she expired before this could be done. Care has been taken to preserve the confidentiality of patient identity

Declarations

Authors’ Affiliations

(1)
Department of Medicine, All India Institute of Medical Sciences

References

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Copyright

© Agrawal et al; licensee BioMed Central Ltd. 2008

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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