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What Is the Cause of Macrocytosis and Dyspnea in an Older Man?

What Is the Cause of Macrocytosis and Dyspnea in an Older Man?

Figure 1
Figure 1
Figure 2
Figure 2

A 78-year-old man presented to the
emergency department with a 3-week
history of progressive shortness of breath
and cough with blood-streaked, yellowish
sputum. The patient had dyspnea on
exertion limited to 2 blocks, 2-pillow
orthopnea, paroxysmal nocturnal dyspnea,
and nocturia. Neither fever nor
chills were present. He had lost 7.2 kg
(16 lb) during the last year.

The medical history included
chronic obstructive pulmonary disease,
hypertension, and type 2 diabetes
mellitus; medications were metformin,
aspirin, metoprolol, and lisinopril.
The patient smoked 2 cigars a day
and denied excessive alcohol consumption.
His brother died at age 39 of a
myocardial infarction; 2 cousins had
thalassemia.

Respiratory distress, pale skin, and
increased jugular venous pressure (jugular
venous column was 11 cm above the
sternal angle) were noted. Lung examination
revealed bilateral crackles halfway
up, with decreased breath sounds and
dullness to percussion. An S3 with a nonradiating,
soft systolic murmur at the left
sternal border was heard. The spleen
was enlarged. The knees had bilateral 2+
pitting edema.

White blood cell count was
2500/?L; hemoglobin, 5 g/dL; hematocrit,
15.8%; platelet count, 128,000/
?L; and mean corpuscular volume,
127 ?m3. Liver function tests and coagulation
profile results were normal.
Vitamin B12, folic acid, methylmalonic
acid, and homocysteine levels were normal.
The peripheral smear (Figures 1
and 2)
demonstrated increased monocytes
with bizarre, stippled, megaloblastic
red blood cells and large platelets.

A chest film revealed cardiomegaly
with pulmonary edema. The echocardiogram
showed normal left ventricular function
with mild tricuspid regurgitation.

High-output congestive heart
failure was diagnosed. The patient
was admitted to the hospital; nitrates,
morphine, angiotensin-converting
enzyme inhibitors, and furosemide
were given for the heart failure, and
packed red blood cells were administered
to increase the hemoglobin
concentration.


Figure 3
Figure 3
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Figure 4

Because of the patient's anemia
and splenomegaly, a hematologist was
consulted. A bone marrow aspirate
(Figures 3 - 5) revealed dyserythropoiet-
ic cells, increased marrow monocytes, a
leftward shift, and ringed sideroblasts.
The total blasts and promyelocytes were
11.5%; refractory anemia with excess
blasts was identified. Six red blood cell
transfusions stabilized the patient's condition;
he was discharged from the
hospital.

Early referral to a hematologist is
warranted for patients with macrocytosis
for which there is no obvious cause. This
patient was referred to a hematologist. He
refused all but symptomatic treatment.

The myelodysplastic syndromes
(Table)—clonal hematologic disorders
characterized by ineffective hematopoiesis—
need to be considered
in the differential diagnosis of patients
with a macrocytic anemia, particularly
those who are older than 50
years. These preleukemic conditions
manifest as slowly developing anemia
that is refractory to standard
therapy and may develop into acute
myelogenous leukemia (AML).
Median age at onset is the seventh
decade of life; approximately 60%
of patients are men.1

  Table — FAB classification of myelodysplastic syndrome subtypes
Diagnosis (relative incidence)   Patient characteristics Peripheral blood characteristics Bone marrow characteristics

Refractory anemia (20% - 30%)   Elderly; anemic < 1% blasts; cytopenia in at least 1 cell line, most often RBCs < 5% blasts; normal or hypercellular marrow

Refractory anemia with ringed sideroblasts (2% - 5%)   Elderly; anemic < 1% blasts; cytopenia in at least 1 cell line, most often RBCs < 5% blasts; >15% of RBC precursors are ringed
sideroblasts

Refractory anemia with excess blasts (30%)   Pancytopenic; dysplastic changes in all 3 lines < 5% blasts; cytopenia of at least 2 cell lines 5% - 20% blasts; normal or hypercellular marrow

Refractory anemia with excess blasts in transformation (25%)   Any age; symptoms of brief duration > 5% blasts; excessive number of monocytes

21% - 30% blasts; normal

or hypercellular marrow


Chronic myelomonocytic leukemia (15% - 20%)   Pancytopenic; dysplastic changes in all 3 lines < 5% blasts; cytopenia of at least 2 cell lines 5% - 20% blasts; normal or hypercellular marrow

FAB, French-American-British system; RBC, red blood cell.
Data from Bennett JM et al. Br J Haematol. 198217; Memorial Sloan-Kettering Cancer Center. 2002.18
Figure 5
Figure 5

Since a subacute form of AML
was first recognized in 1949,2-4 many
terms—including refractory anemia,
preleukemia, oligoblastic leukemia,
smoldering acute leukemia, refractory
dysmyelopoietic dysplasia, and
hematopoietic dysplasia—have been
used to describe the syndromes of
proliferative hematopoietic failure
that often progress to AML.5-7

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