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Chronic lymphocytic leukemia
Symptoms and signs
The disease usually takes an indolent course with median survival
reaching up to 10 years in untreated individuals.
Asymptomatic (40%)
The usual presentation is without symptoms
and lymphocytosis is only discovered on examination of the blood
picture. With the use of routine blood testing, the number of CLL
patients who are asymptomatic at diagnosis has increased. Some
patients may remain asymptomatic for long periods of time. The disease may progress to generalized lymphadenopathy and
splenomegaly. The peripheral blood picture reveals a high white cell
count (usually above 100,000) that expresses absolute lymphocytosis.
It may also show associated pancytopenia.
Fatigue
The most common complaint is fatigue, but this is generally not
severe.
Lymph node enlargement
The most consistent abnormal
finding on physical examination is lymphadenopathy. Most patients have noted painless swelling of
lymph nodes, often in the cervical area (but also
at times in any other lymph node-bearing site),
that spontaneously waxes and wanes but does not
altogether disappear. The size of enlarged
nodes may be as small as a few millimeters in diameter or as large
as an orange.
Characteristically,
enlarged nodes in CLL are firm, rounded, discrete,
nontender, and freely mobile upon palpation,
although exceptions to these generalizations
are encountered, particularly when nodes
have grown rapidly.
Occasionally, several
enlarged nodes in the same anatomic site (cervical
triangle, axilla, or femora–inguinal areas)
may become confluent with each other, forming
large spherical lymphoid masses. New lymph
nodes may appear, sometimes in places other
than the usual lymph node- bearing sites, such as
over the sacrum or the thorax.
Infections
Bacterial infections, such as pneumonia, are more common in
patients who present with advanced-stage disease. Infections
secondary to opportunistic organisms, particularly herpes zoster,
may occur. An exaggerated skin reaction to a bee sting or an insect
bite is frequent (Well's syndrome).
Splenomegaly (30%)
Splenomegaly may occur, but massive splenomegaly is usually only
seen in patients with end-stage disease. Splenic infarction is rare.
It
may be palpably enlarged in 30% to 40% of
cases. The extent of enlargement varies from an
organ barely palpable upon deep inspiration to
one so large as to occupy the entire left side of
the abdomen and pelvis, to cross the midline and
encroach upon the right side of the abdomen. As
is the case with enlarged lymph nodes, an
enlarged spleen in CLL is usually painless and
upon palpation is nontender with a sharp edge
and a smooth, firm surface. Painful or infarcted
splenic enlargement is an unusual presenting
feature for CLL.
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Hepatomegaly (20%)
Hepatomegaly occurs less frequently than splenomegaly. Liver Enlargement of the liver may be
noted at the time of initial diagnosis of CLL in
approximately 20% of cases. The liver in CLL
generally is not greatly enlarged, ranging from 2
to 6 cm below the right costal margin, with a
span of dullness on percussion of 10 to 16 cm.
Upon palpation, the liver is usually nontender
and firm, with a smooth surface.
In addition to palpably enlarged peripheral
lymph nodes, liver, and spleen, virtually any other
lymphoid tissue in the body may be enlarged at
diagnosis, such as Waldeyer ring or the tonsils.
Organ infiltration (<5%)
Infiltration with CLL cells may occur in any
organ; at the time of diagnosis, skin lesions are
the most obvious, but are seen in less than 5% of
cases. In contrast to lymphoma, gastrointestinal
mucosal involvement is rarely seen in CLL. Similarly,
meningeal leukemia is unusual in CLL at
the time of initial presentation; if present, is usually seen
in patients with refractory disease.
B symptoms
B symptoms are quite uncommon in patients with CLL. In contrast
to the situation in lymphoma, fever in the absence of infection is
rare in CLL.
Autoimmune hemolytic anemia and/or thrombocytopenia can occur in
patients with any stage of CLL.
Autoimmune Complications
When autoantibodies are present in CLL, they are usually targeted
against hematopoietic cells, resulting in AIHA, immune
thrombocytopenia, immune-mediated granulocytopenia, and pure red
cell aplasia.
AIHA is the most frequently occurring of these.
Several factors indicate that antibodies against blood cell antigens
are not produced by the leukemic clone. These autoantibodies are
polyclonal and are usually immunoglobulin G. The severity of the
autoimmune phenomenon does not necessarily correlate with the
severity of CLL, and such events may develop in patients whose
disease is responding to therapy with fludarabine.
Prednisone (P) is
the most commonly used treatment for autoimmune complications, with
high initial response rates. Relapses are not uncommon. Cyclosporin
A is another effective therapy and can produce good results, even in
steroid-refractory patients. The monoclonal antibodies rituximab
and alemtuzumab have also been used in some patients in whom
standard therapy fails and have produced responses.
Transformation
Transformation of CLL to diffuse large cell lymphoma (Richter's
syndrome) carries a poor prognosis.
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