What are the “classic” clinical and basic laboratory features of each of the common hematologic malignancies? (“How do I prove the diagnosis?”)
What is the natural history of each disease entity without therapy?
What is the goal of treatment for each disease entity (cure, prolongation of survival, or purely palliation)?
Which modalities may be employed for each disease entity (chemotherapy, immunotherapy, radiation, stem cell transplant)?
What is the prognosis with therapy? How can the prognosis estimate be refined?
What needs to be done to prepare the patient for therapy (to communicate risks and benefits and to reduce toxicity)?
How do we assess the response?
How do we monitor the patient after completion of therapy, both early on (for relapse) and later (for delayed toxicities of therapy)?
Practitioners need to be familiar with the broad classes of hematologic malignancy (acute and chronic leukemias, aggressive and indolent lymphomas, myeloma, myeloproliferative neoplasms, and myelodysplastic syndromes). They should also be familiar with the side effects of therapy, for example, acute and chronic cardiotoxicity from anthracyclines, long-lasting immunosuppression from purine analogs, and the expected timing of neutropenia (Table 179-1). He or she should also be comfortable managing a number of hematologic emergencies, which are described in the following chapter. The specifics of diagnosis and treatment can be both subtle and complex. Subspecialty consultation with a hematologist or oncologist is essential in caring for a patient who is suspected or known to suffer these conditions.
Table 179-1 Common Antineoplastic Agents and Their Side Effects |Favorite Table|Download (.pdf)
Table 179-1 Common Antineoplastic Agents and Their Side Effects
|Chemotherapy and targeted therapy|
|All-trans retinoic acid (ATRA) and arsenic trioxide (ATO)||Differentiating agents||Acute promyelocytic leukemia|
- Life-threatening hyperleukocytosis and the differentiation syndrome, marked by weight gain and edema, progressing to dyspnea, interstitial pulmonary infiltrates, pleural and/or pericardial effusions, unexplained fevers, and acute renal insufficiency.
- Other toxicities unique to differentiating agents include pseudotumor cerebri with ATRA and dangerously prolonged QT intervals and hyperglycemia with ATO. If a patient on ATO presents with syncope or palpitations and the absolute QT interval is > 500 msec, ATO should be withheld, electrolytes repleted, and QT-prolonging drugs stopped.
|Dexamethasone 10 mg IV twice a day should be started at the earliest sign of differentiation syndrome, even if other etiologies like sepsis or primary CHF are possible. In patients with acute renal failure or requiring ICU admission for respiratory distress, ATRA or ATO should be temporarily held.|
|Anagrelide||A prostaglandin synthetase inhibitor, specifically inhibits platelet production but occasionally causes mild anemia||Myeloproliferative neoplasms||Palpitations, CHF, noncardiac edema, diarrhea, abdominal pain, headache, bleeding, anemia||In the MRC PT-1 trial, aspirin + hydroxyurea were compared to aspirin + anagrelide treatment for ET. There were more arterial events and fewer venous thromboses in the anagrelide arm. Anagrelide increased progression to marrow fibrosis and conferred a higher risk of ...|