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6 Part I: Clinical Evaluation of the Patient Chapter 1: Initial Approach to the Patient: History and Physical Examination 7
number of prior pregnancies and an accurate assessment of the extent with methemoglobinemia, either hereditary or acquired; sulfhemoglo-
of menstrual blood loss. Semiquantification can be obtained from esti- binemia; abnormal hemoglobins with low oxygen affinity; and primary
mates of the number of days of heavy bleeding (usually <3), the num- and secondary polycythemia. Cyanosis of the ears or the fingertips may
ber of days of any bleeding (usually <7), number of tampons or pads occur after exposure to cold in individuals with cryoglobulins or cold
used (requirement for double pads suggests excessive bleeding), degree agglutinins.
of blood soaking, and clots formed, and inquiries such as, “Have you Itching may occur in the absence of any visible skin lesions in
experienced a gush of blood when a tampon is removed?” However, an Hodgkin lymphoma and may be extreme. Mycosis fungoides or other
objective distinction between menorrhagia (loss of more than 80 mL lymphomas with skin involvement may also present as itching. A sig-
blood per period) and normal blood loss can best be made by a visual nificant number of patients with polycythemia vera will complain of
assessment technique using pictorial charts of towels or tampons. Men- itching after bathing.
6
orrhagia may occur in patients with bleeding disorders. Petechiae and ecchymoses are most often seen in the extremities in
patients with thrombocytopenia, nonthrombocytopenic purpura, or
Back and Extremities acquired or inherited platelet function abnormalities and von Wille-
Back pain may accompany acute hemolytic reactions or be a result brand disease. Unless secondary to trauma, these lesions usually are
of involvement of bone or the nervous system in acute leukemia or painless; the lesions of psychogenic purpura and erythema nodosum are
aggressive lymphoma. It is one of the most common manifestations of painful. Easy bruising is a common complaint, especially among women,
myeloma. and when no other hemorrhagic symptoms are present, usually no
Arthritis or arthralgia may occur with gout secondary to increased abnormalities are found after detailed study. This symptom may, how-
uric acid production in patients with hematologic malignancies, espe- ever, indicate a mild hereditary bleeding disorder, such as von Wille-
cially acute lymphocytic leukemia in childhood, myelofibrosis, myel- brand disease or one of the platelet disorders. Infiltrative lesions may
odysplastic syndrome, and hemolytic anemia. They also occur in the occur in the leukemias (leukemia cutis) and lymphomas (lymphoma
plasma cell dyscrasias, acute leukemias, and sickle cell disease without cutis) and are sometimes the presenting complaint. Monocytic leukemia
evidence of gout, and in allergic purpura. Arthritis may accompany has a higher frequency of skin infiltration than other forms of leukemia.
hemochromatosis, although the association has not been carefully Necrotic lesions may occur with intravascular coagulation, purpura ful-
established. In the latter case the arthritis starts typically in the small minans, and warfarin-induced skin necrosis, or rarely with exposure to
joints of the hand (second and third metacarpal joints), and episodes cold in patients with circulating cryoproteins or cold agglutinins.
of acute synovitis may be related to deposition of calcium pyrophos- Leg ulcers are a common complaint in sickle cell anemia and occur
phate dehydrate crystals. Hemarthroses in patients with severe bleeding rarely in other hereditary anemias.
disorders cause marked joint pain. Autoimmune diseases may present
as anemia and/or thrombocytopenia, and arthritis appears as a later DRUGS AND CHEMICALS
manifestation. Shoulder pain on the left may be a result of infarction
of the spleen and on the right of gall bladder disease associated with Drugs
chronic hemolytic anemia such as hereditary spherocytosis. Bone pain Drug therapy, either self-prescribed or ordered by a physician, is
may occur with bone involvement by the hematologic malignancies; extremely common in our society. Drugs often induce or aggravate
it is common in the congenital hemolytic anemias, such as sickle cell hematologic disease, and it is therefore essential that a careful history
anemia, and may occur in myelofibrosis. In patients with Hodgkin lym- of drug ingestion, including beneficial and adverse reactions, should
phoma, ingestion of alcohol may induce pain at the site of any lesion, be obtained from all patients. Drugs taken regularly, including nonpre-
including those in bone. Edema of the lower extremities, sometimes scription medications, often become a part of the patient’s way of life
unilateral, may occur because of obstruction to veins or lymphatics by and are forgotten or are not recognized as “drugs.”
lymphomatous masses or from deep venous thrombosis. The latter can Agents such as aspirin, laxatives, tranquilizers, medicinal iron,
also cause edema of the upper extremities. vitamins, other nutritional supplements, and sedatives belong to this
category. Furthermore, drugs may be ingested in unrecognized form,
Skin such as antibiotics in food or quinine in tonic water. Specific, persis-
Skin manifestations of hematologic disease may be of great importance; tent questioning, often on several occasions, may be necessary before a
they include changes in texture or color, itching, and the presence of complete history of drug use is obtained. It is very important to obtain
specific or nonspecific lesions. The skin in iron-deficient patients may detailed information on alcohol consumption from every patient. The
become dry, the hair dry and fine, and the nails brittle. In hypothy- four “CAGE” questions—about needing to cut down, being annoyed
roidism, which may cause anemia, the skin is dry, coarse, and scaly. by criticism, having guilt feelings, and requiring a drink as a morning
Jaundice may be apparent with pernicious anemia or congenital or eye-opener—provide an effective approach to the history of alcohol use.
acquired hemolytic anemia. The skin of patients with pernicious anemia Patients should also be asked about the use of recreational drugs. The
is said to be “lemon yellow” because of the simultaneous appearance of use of “alternative medicines” and herbal medicines is common, and
jaundice and pallor. Jaundice may also occur in patients with hemato- many patients will not consider these medications or may actively with-
logic malignancies, especially lymphomas, as a result of liver involve- hold information about their use. Nonjudgmental questioning may be
ment or biliary tract obstruction. Pallor is a common accompaniment successful in identifying agents in this category that the patient is tak-
of anemia, although some severely anemic patients may not appear pale. ing. Some patients equate the term “drugs,” as opposed to “medicines,”
Erythromelalgia may be a troublesome complication of polycythemia with illicit drugs. Establishing that the examiner is interested in all
vera. Patchy plaques or widespread erythroderma occur in cutaneous forms of ingestants—prescribed drugs, self-remedies, alternative reme-
T-cell lymphoma (especially Sézary syndrome) and in some cases of dies, etcetera—is important to ensure getting the information required.
chronic lymphocytic leukemia or lymphocytic lymphoma. The skin is
often involved, sometimes severely, in graft-versus-host disease follow- Chemicals
ing hematopoietic cell transplantation. Patients with hemochromatosis In addition to drugs, most people are exposed regularly to a variety of
may have bronze or grayish pigmentation of the skin. Cyanosis occurs chemicals in the environment, some of which may be potentially harmful
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