Correspondence

Case 40-2000: Gastric Cancer in a Young Woman

N Engl J Med 2001; 344:1949-1950June 21, 2001

Article

To the Editor:

We were surprised that the diagnosis of pernicious anemia in Case 40-2000 (Dec. 28 issue)1 was based only on a mean corpuscular volume of 104 μm³, the presence of atrophic gastritis, and a serum B₁₂ level of 115 pg per milliliter. For a diagnosis of pernicious anemia, there must be antibodies to intrinsic factor in the serum or evidence of inadequate absorption of vitamin B₁₂ that is correctable by the administration of oral intrinsic factor (as determined by the Schilling test). These are the gold standards.

In the case discussed, there is no description of the peripheral-blood smear, which is important because macrocytosis, measured only electronically, in the absence of anemia is usually due to rouleaux or agglutinins. Furthermore, the patient's age of 38 years is lower than that of most patients with pernicious anemia; there is no report of megaloblastic maturation of the gastrointestinal mucosa in the pathological specimen; and the single low (but not markedly decreased) level of vitamin B₁₂ is higher than that commonly seen in pernicious anemia. The absence of any neurologic abnormality would also be distinctly unusual in a patient with pernicious anemia.

Fred Rosner, M.D.
Hans W. Grünwald, M.D.
Mount Sinai Services at Queens Hospital Center, Jamaica, NY 11432

1 References

1. 1

   Case Records of the Massachusetts General Hospital (Case 40-2000). N Engl J Med 2000;343:1951-1958
   Full Text | Web of Science | Medline

To the Editor:

This case report covers many details of the possible causes of the patient's illness, and includes a lengthy discussion of Helicobacter pylori infection, pernicious anemia, and vitamin B₁₂ metabolism. In addition, approximately one and a half pages are used for histopathological illustrations. Sadly absent is any discussion of the pastoral considerations essential to the care of a young patient who had a disease so likely to be rapidly fatal (as indeed it proved). Did she have any family support? Were any options other than radical surgical resection presented to her? Was anything useful achieved by intervention, or did it only add to her worsening health problems?

Students and postgraduate trainees read the Case Records of the Massachusetts General Hospital with great attention. In my view, it is not sufficient, in a lengthy exposition occupying eight Journal pages, to disregard these important features. We are constantly criticized as being insufficiently concerned about holistic care and as being out of touch with real-life issues; this case seems to demonstrate this all too clearly. In summary, a young woman with a dreadful and rapidly fatal cancer was treated unsuccessfully by radical surgery and chemotherapy. She died after four months, and any beneficial effect was clearly only marginal. In such a lengthy article, a wider, more discursive (and self-questioning) discussion would have greatly added to the educational value of the report, even if it had meant reducing by half the six handsomely colored histopathological figures. When we cannot cure patients, we have a special responsibility to pay particular attention to their palliative and pastoral needs.

Jeffrey S. Tobias, M.D.
University College London Hospitals, London W1T 3AA, United Kingdom

Author/Editor Response

The author replies:

To the Editor: Drs. Rosner and Grünwald suggest that the presence of antibodies against intrinsic factor or an increase in vitamin B₁₂ absorption after the administration of intrinsic factor (the Schilling test) is required to make the diagnosis of pernicious anemia. The results of a Schilling test can be diagnostic, but the test was not performed in this case. The presence of antibodies against intrinsic factor would be diagnostic in most cases, since they are quite specific for pernicious anemia. However, this is not a very sensitive method of assessment (since the antibodies are present in only 50 to 60 percent of patients with pernicious anemia),1 and this test was not done. Evidence of autoimmune gastritis in a biopsy specimen (as in this case) or the absence of intrinsic factor in a stimulated gastric-juice assay are definitive test results, but such tests are rarely needed. A blood smear is very helpful in making a diagnosis of megaloblastic anemia2 but was not available. Although this patient was young to have pernicious anemia, vitamin B₁₂ levels similar to hers and the absence of neurologic symptoms are common findings in pernicious anemia. In one study in which vitamin B₁₂ deficiency was defined by a response to vitamin B₁₂ treatment, 38 percent of patients had vitamin B₁₂ levels between 100 and 200 pg per milliliter, and 28 percent had neurologic abnormalities.2

Christopher L. Carpenter, M.D.
Beth Israel Deaconess Medical Center, Boston, MA 02215

2 References

1. 1

   Fairbanks VF, Lennon VA, Kokmen E, Howard FM Jr. Tests for pernicious anemia: serum intrinsic factor blocking antibody. Mayo Clin Proc 1983;58:203-204
   Web of Science | Medline

2. 2

   Stabler SP, Allen RH, Savage DG, Lindenbaum J. Clinical spectrum and diagnosis of cobalamin deficiency. Blood 1990;76:871-881
   Web of Science | Medline

Author/Editor Response

The editor of the Case Records replies:

To the Editor: In response to Dr. Tobias: over the years, the Case Records have been criticized in the Correspondence section of the Journal for not including consideration of a number of aspects of patient care and health policy. These have included the following: analysis of the patient's hospital bills, with evaluation of the cost effectiveness of the various diagnostic tests and procedures that were performed; discussion of the social background of each patient; psychiatric aspects of his or her illness; and the patient's occupation and its possible influence on his or her disease.

The answer to all these criticisms is not difficult. The Case Records were founded at the dawn of the 20th century to teach physicians about the correlation between the clinical and pathological features of various diseases, with an emphasis on differential diagnosis. The Journal limits the length of the printed versions of these live exercises, necessitating a marked reduction in the length of the transcribed live discussion. It is therefore difficult to consider other very important topics, such as those mentioned by Dr. Tobias. Those topics certainly deserve to be discussed in detail in other venues, but they are not a part of the educational mission of one of the very few current series of medical reports devoted to the art and science of diagnosis on a case-by-case basis.

Robert E. Scully, M.D.
Massachusetts General Hospital, Boston, MA 02114-2696