FARR Hematology/Oncology Flashcards
Four causes of microcytic anemia.
TICS—Thalassemia, Iron deficiency, anemia of Chronic disease, and Sideroblastic anemia.
An elderly man with hypochromic, microcytic anemia is asymptomatic. Diagnostic tests?
Fecal occult blood test and sigmoidoscopy; suspect colorectal cancer.
Precipitants of hemolytic crisis in patients with G6PD deficiency.
Sulfonamides, antimalarial drugs, fava beans.
The most common inherited cause of hypercoagulability.
Factor V Leiden mutation.
The most common inherited bleeding disorder.
von Willebrand’s disease.
The most common inherited hemolytic anemia.
Hereditary spherocytosis.
Diagnostic test for hereditary spherocytosis.
Osmotic fragility test.
Pure RBC aplasia.
Diamond-Blackfan anemia.
Anemia associated with absent radii and thumbs, diffuse hyperpigmentation, café au lait spots, microcephaly, and pancytopenia.
Fanconi’s anemia.
Medications and viruses that lead to aplastic anemia.
Chloramphenicol, sulfonamides, radiation, HIV, chemotherapeutic agents, hepatitis, parvovirus B19, EBV.
How to distinguish polycythemia vera from 2° polycythemia.
Both have ↑ hematocrit and RBC mass, but polycythemia vera should have normal O2 saturation and low erythropoietin levels.
Thrombotic thrombocytopenic purpura (TTP) pentad?
“FAT RN”: Fever, Anemia, Thrombocytopenia, Renal dysfunction, Neurologic abnormalities.
HUS triad?
Anemia, thrombocytopenia, and acute renal failure.
Treatment for TTP.
Emergent large-volume plasmapheresis, corticosteroids, antiplatelet drugs.
Treatment for idiopathic thrombocytopenic purpura (ITP) in children.
Usually resolves spontaneously; may require IVIG and/or corticosteroids.
Which of the following are ↑ in DIC: fibrin split products, D-dimer, fibrinogen, platelets, and hematocrit.
Fibrin split products and D-dimer are elevated; platelets, fibrinogen, and hematocrit are ↓.
An eight-year-old boy presents with hemarthrosis and ↑ PTT with normal PT and bleeding time. Diagnosis? Treatment?
Hemophilia A or B; consider desmopressin (for hemophilia A) or factor VIII or IX supplements.
An eight-year-old boy presents with hemarthrosis and ↑ PTT with normal PT and bleeding time. Diagnosis? Treatment?
Hemophilia A or B; consider desmopressin (for hemophilia A) or factor VIII or IX supplements.
A 14-year-old girl presents with prolonged bleeding after dental surgery and with menses, normal PT, normal or
↑ PTT, and ↑ bleeding time. Diagnosis? Treatment?
von Willebrand’s disease; treat with desmopressin, FFP, or cryoprecipitate.
A 60-year-old African-American man presents with bone pain. Workup for multiple myeloma might reveal?
Monoclonal gammopathy, Bence Jones proteinuria, “punched-out” lesions on x-ray of the skull and long bones.
Reed-Sternberg cells.
Hodgkin’s lymphoma.
A 10-year-old boy presents with fever, weight loss, and night sweats. Exam shows an anterior mediastinal mass. Suspected diagnosis?
Non-Hodgkin’s lymphoma.
Microcytic anemia with ↓ serum iron, ↓ total iron-binding capacity (TIBC), and normal or ↑ ferritin.
Anemia of chronic disease.
Microcytic anemia with ↓ serum iron, ↓ ferritin, and ↑ TIBC.
Iron deficiency anemia.
An 80-year-old man presents with fatigue, lymphadenopathy, splenomegaly, and isolated lymphocytosis. Suspected diagnosis?
Chronic lymphocytic leukemia (CLL).
The lymphoma equivalent of CLL.
Small lymphocytic lymphoma.
A late, life-threatening complication of chronic myelogenous leukemia (CML).
Blast crisis (fever, bone pain, splenomegaly, pancytopenia).
Auer rods on blood smear.
Acute myelogenous leukemia (AML).
AML subtype associated with DIC.
M3.
Electrolyte changes in tumor lysis syndrome.
↓ Ca2+, ↑ K+, ↑ phosphate, ↑ uric acid.
Treatment for AML M3.
Retinoic acid.
A 50-year-old man presents with early satiety, splenomegaly, and bleeding. Cytogenetics show t(9,22). Diagnosis?
CML.
Heinz bodies?
Intracellular inclusions seen in thalassemia, G6PD deficiency, and postsplenectomy.
An autosomal-recessive disorder with a defect in the GPIIbIIIa platelet receptor and ↓ platelet aggregation.
Glanzmann’s thrombasthenia.
Virus associated with aplastic anemia in patients with sickle cell anemia.
Parvovirus B19.
A 25-year-old African-American man with sickle cell anemia has sudden onset of bone pain. Management of pain crisis?
O2, analgesia, hydration, and, if severe, transfusion.
A significant cause of morbidity in thalassemia patients. Treatment?
Iron overload; use deferoxamine.
Heparin:
↑ PTT, activates antithrombin III and affects the intrinsic path- way, and ↓ fibrinogen levels; protamine sulfate is the antidote.
Warfarin:
↑ PT, inhibits vitamin K and affects the extrinsic pathway, and is teratogenic, since its small size allows it to cross the placenta. Vitamin K is the antidote. Goal INR of 2.0–3.0 (2.5–3.5 in patients with mechanical valves).
Enoxaparin
(low-molecular-weight heparin [LMWH]): Inhibits factor Xa and does not have to be monitored; dosing is once or twice daily.
Heparin-to-warfarin conversion is necessary because
warfarin inhibits proteins C and S before other vitamin K–dependent factors (II, VII, IX, and X), lead- ing to a transient period of paradoxical hypercoagulability before proper an- ticoagulation.
Hemophilia
A deficiency of a clotting factor that leads to a bleeding diathesis.
Hemophilia HISTORY/PE
I Presents with spontaneous hemorrhage into the tissues and joints that, if left untreated, can lead to arthropathy and joint destruction.
I Spontaneous intracerebral hemorrhages, renal and retroperitoneal bleed- ing, and GI bleeding may also be seen.
I Mild cases may have major hemorrhage after surgery or trauma but are otherwise asymptomatic.
von Willebrand’s Disease (vWD)
Hx/PE
Presents with easy bruising, mucosal bleeding (e.g., epistaxis, oral bleeding), menorrhagia, and postincisional bleeding. Platelet dysfunction is not severe enough to produce petechiae. Symptoms worsen with ASA use.
Platelet count and PT are normal, but a prolonged aPTT may be seen as
a result of factor VIII deficiency.
Hypercoagulable States Hx/PE
Presents with recurrent thrombotic complications, including DVT, pul- monary embolism, arterial thrombosis, MI, and stroke. Women may have recurrent miscarriages.
Although patients may have no recognizable predisposing factors, they usually have one or more of the causative factors outlined in Table 2.7-2. They may also have a
Disseminated Intravascular Coagulation (DIC)
A common disorder among hospitalized patients, second only to liver disease as a cause of acquired coagulopathy. It is caused by deposition of fibrin in small blood vessels, leading to thrombosis and end-organ damage. Depletion of clotting factors and platelets leads to a bleeding diathesis. May be associ- ated with almost any severe illness.
Thrombotic Thrombocytopenic Purpura (TTP)
Part of a spectrum of diseases that includes hemolytic-uremic syndrome (HUS) and HELLP syndrome (see the Obstetrics chapter); thought to be due to platelet microthrombi that block off small blood vessels, leading to end- organ ischemia and dysfunction. RBCs are fragmented by contact with the microthrombi, leading to hemolysis (microangiopathic hemolytic anemia). The cause of initial microthrombus formation is unknown but may be infec- tious (bacterial toxins), drug related, autoimmune, or idiopathic.
TPP Hx/Pe
A clinical syndrome characterized by five signs/symptoms: low platelet count, microangiopathic hemolytic anemia, neurologic changes (delirium, seizure, stroke), impaired renal function, and fever.
Treat with corticosteroids to ↓ the formation of microthrombi along with plasma replacement and plasmapheresis.
Overlapping conditions of TPP are
I HUS: Characterized by renal failure, hemolytic anemia, and low plate- lets. Severe elevations in creatinine are more typical of HUS than of
TTP.
I HELLP syndrome: Affects pregnant women, often occurring in con-
junction with preeclampsia (see the Obstetrics chapter).
I DIC: Distinguished from TTP by prolonged PT and aPTT.
Idiopathic Thrombocytopenic Purpura (ITP)
A relatively common cause of thrombocytopenia. IgG antibodies are formed against the patient’s platelets. Bone marrow production of platelets is ↑, with ↑ megakaryocytes in the marrow. The most common immunologic disorder in women of childbearing age. May be acute or chronic.
Idiopathic Thrombocytopenic Purpura (ITP)
present with no systemic symptoms
associated with lymphoma, leuke- mia, SLE, HIV, and HCV.
Acute (2-6years) or Chronic (20-40years with infection)
A diagnosis of exclusion
Iron deficiency anemia in an elderly patient could be due to
colorectal cancer and needs to be evaluated to rule out malignancy.
Causes of microcytic anemia—
TICS
Thalassemia
Iron deficiency Chronic disease Sideroblastic anemia
APLASTIC ANEMIA
A rare condition caused by failure of blood cell production due to destruc- tion of bone marrow cells. It may be hereditary, as in Fanconi’s anemia; may have an autoimmune or a viral etiology (e.g., HIV, parvovirus B19); or may result from exposure to toxins (e.g., drugs, cleaning solvents) or radiation.
Polycythemia
Patients present with “hyperviscosity syndrome,” which consists of easy bleeding/bruising, blurred vision, neurologic abnormalities, plethora, pruritus (especially after a warm bath), hepatomegaly, splenomegaly, and CHF.
Phlebotomy relieves symptoms
Porphyria
I Signs and symptoms vary with the type of porphyria. In general, however, porphyrias are characterized by a combination of photodermatitis, neu- ropsychiatric complaints, and visceral complaints that typically take the form of a colicky abdominal pain and seizures.
I Physical exam reveals tachycardia, skin erythema and blisters, areflexia, and a nonspecific abdominal exam.
the most common child- hood malignancy.
ALL
ALL and AML are treated primarily with
chemotherapeutic agents, al- though transfusions, antibiotics, and colony-stimulating factors are also used. Patients with unfavorable genetics or those who do not achieve re- mission may be candidates for bone marrow transplantation.
I Prior to therapy, patients should be well hydrated, and if their WBC counts are high, they may be started on allopurinol to prevent hyperuricemia and renal insufficiency resulting from blast lysis (tumor lysis syndrome).
I Leukostasis syndrome may be treated with hydroxyurea +/– leukapheresis to rapidly ↓ WBC count.
AML type (FAB) M3—acute promyelocytic leukemia (APL)—has a good prognosis because
it is responsive to all-trans-retinoic acid (ATRA) therapy.
Philadelphia chromosome t(9,22).
CML
Leukocyte alkaline phosphatase is low; LDH, uric acid, and B12 levels are elevated. CML or CLL
CML
Most NHLs (almost 85%) are of xxx-cell origin. NHL is the most common xxx neoplasm and is xxx times more common than Hodgkin’s lymphoma.
B
hematopoietic
five
