Hematology/Oncology Flashcards
Chronic Lymphocytic Leukemia
- Clinical
- Diagnostic
- Prognostic
- Complicatons
- Treatment
- Treatment: Rituximab (anti-CD20)
Indications for treatment of CLL
Patients with CLL have a median survival of 10 years, and treatment is not beneficial if a patient is asymptomatic
- Progressive bone marrow failure (causing cytopenias)
- Massive lymphadenopathy/splenomegaly
- Presence of severe B symptoms (night sweats, weight loss)
Etiology of infections in sickle cell
- Pneumonia
- Osteomyelitis/septic arthritis
- Bacteremia/sepsis
- Meningitis
Laboratory abnormalities during vaso-occlusive pain crisis due to sickle cell
Anemia
Leukocytosis
SIgns of hemolysis (elevated LDH and indirect bilirubin)
Multiple myeloma
- Pathophysiology
- Manifestations
- Laboratory
- Radiology
Etiology of renal failure in multiple myeloma (MM)
- Myeloma cast nephropathy: Monoclonal light chains clog renal tubules, causing intratubular cast formation and toxicity - renal tubular injury
- Amyloidosis (glomerular injury - nephrotic syndrome)
- Monoclonal immunoglobulin deposition disease (glomerular injury - nephrotic syndrome)
Disseminated intravascular coagulation
Most common coagulopathy in malignancy (gastric, breast, lung cancer)
Thrombocytopenia
Decreased fibrinogen
Increased INR
-Anemia due to hemolysis from microangiopathic hemolytic anemia (MAHA)
Hyposegmented/hypogranulated neutrophils
Characteristic of myelodysplastic syndrome
Myelodysplastic syndrome
- Epidemiology
- Manifestations
- Diagnosis
- Treatment
Pernicious anemia
Anti-intrinsic factor antibodies
B12 deficiency
Increased methylmalonic acid
Macrocytic anemia
Hypersegmented neutrophils
Rouleaux formation
Characteristic of multiple myeloma
Smudge cells (fragile but mature lymphocytes)
Characteristic of chronic lymphocytic leukemia
Hypersegmented neutrophils
Characteristic of B12 deficiency
Lymphoblasts
Seen in acute lymphoblastic leukemia
Auer rods
Seen in acute promyelocytic leukemia (APML)
Teardrop shaped red blood cells
Seen in myelofibrosis or beta thalessemia (especially after splenectomy)
Febrile nonhemolytic transfusion reactions (time course)
1-6 hours
Benign
Hereditary spherocytosis
Increased risk of bilirubin gallstones
Treat with blood transfusions and folic acid supplementation, consider splenectomy
Secondary thrombocytosis
Caused by cytokines promoting platelet production
Usually driven by an inflammatory state (infection, recent surgery, malignancy)
Often occurs after splenectomy: Typically platelet counts normalize after weeks, but patients may have thrombocytosis for months or years.
Initial screening test for a non-African female with a family history of anemia, concerned for hemogloninopathy
Complete blood count
Hemophilia A & B
- Inheritance
- Clinical features
- Laboratory findings
- Treatment
Severe hemophilic arthropathy
Hemophilic arthropathy
Iron study findings in microcytic/hypochromic anemias
- Iron deficiency anemia
- Thalassemia
- Anemia of chronic disease
- Sideroblastic anemia
Coagulation cascade pathway
Vitamin K deficiency
- Risk factors
- Clinical features
- Laboratory findings
Obstructive sleep apnea
- Pathophysiology
- Symptoms
- Sequelae
Drugs that cause folic acid deficiency
Anti-epileptics: Phenytoin, primidone, phenobarbital
Impair absorption of folic acid in the small intestine
Trimpethoprim: Inhibits dihydrofolate reductase and in high doses can cause megaloblastic pancytopenia
Methotrexate: Inhibits dihydrofolate reductase (treat with leucovorin – folinic acid)
Drug that causes B6 deficiency
Isoniazid
Treatment of deep venous thrombosis
Immune deficiency in asplenia
Defects in antibody response, antibody-mediated phagocytosis/complement activation
At risk for fulminant infection with encapsulated bacteria (Strep pneumo, H. influenzae)
Diseases characterized by complement deficiency
Systemic lupus erythematosus
Antiphospholipid antibody syndrome
(Increased risk of infection with encapsulated bacteria)
Impaired B cell isotype switching
Seen in patients with common variable immunodeficiency
Normal B cell numbers but significantly reduced Ig subtypes
Risk for recurrent infection (e.g., sinopulmonary, GI) and antoimmune disease
Leukocyte adhesion deficiency
Impaired chemotaxis
Recurrent bacterial infections of skin and mucosa
Chronic granulomatous disease
Impaired oxidative burst
Recurrent bacterial or fungal infections due to catalase-producing organisms (e.g. Asperigillus nidulans, Staphylococcus aureus)
Thrombotic thrombocytopenic purpura
- Pathophysiology
- Clinical features
- Management
Treatment for cancer-related anorexia/cachexia syndrome (CACS)
- Progresterone analogues (megestrol acetate and medroxyprogesterone acetate)
- Corticosteroids
Progesterone analoges prefereed due to decreased incidence of side effects
RBC agglutination
Seen in Waldenstrom’s macroglobulinemia
Plasma cell neoplasm characterized by excessive monoclonal IgM, end-organ damage, and >10% clonal lymphocytes by bone marrow biopsy. Hyperviscosity syndrome, neuropathy, and cryoglobulinemia are common consequences of elevated serum IgM.
Waldenstrom Macroglobulinemia versus Multiple Myeloma
- Major manifestations
- Monoclonal antibody
- Peripheral smear
- Bone marrow biopsy
Clinical features of type 2 heparin-induced thrombocytopenia
- Clinical signs
- Diagnostic evaluation
- Therapy
HIT is a life-threatening ocmplication of heparin therapy. HIT is antibody-mediated and causes a mild to moderate thrombocytopenia with minimal bleeding risk. Venous and arterial thrombotic risk is significant, as high as 50% in untreated HIT.
All heparin products should be discontinued immediately.
Warfarin is used for anticoagulation maintenance in HIT but only after the patient has received another anticoagulant and the platelet count is >150,000. Initial treatment with warfarin is contraindicated in HIT as it rapidly lowers protein C levels, which may further increase the risk of thrombus.
Anemia of prematurity
- Etiology
- Clinical manifestations
- Laboratory findings
- Treatment
After birth, circulating EPO decreases due to increased oxygen concnetration in tissue. Decreased EPO causes decreased reticulocyte production in bone marrow.
Target cells (and hypochromic microcytic RBCs)
Alpha or beta thalassemia
Bite cells (and Heinz bodies)
Characteristic of glucose-6-phosphate dehydrogenase deficiency
Sickle cell anemia
(Infants are protected by fetal hemoglobin in first 4-6 months of life)
Immune thrombocytopenia
- Clinical presentation
- Laboratory findings
- Treatment: Children, adults
Children usually recover spontaneously within 6 months and require only observation, regardless of platelet count. Children with bleeding should receive IvIG or glucocorticoids.
Positive Coomb’s test
Warm, antibody-type, autoimmune hemolytic anemia
Most commonly seen in women with some underlying disease affective the immune system (e.g., lymphoid neoplasm, collagen vascular disease, congenital immunodeficiency diseases).
JAK2 mutation
Associated with myeloproliferative diseases, particularly polycythemia vera (PV)
Serum protein electrophoresis
Can detect elevated levels of monoclonal proteins
Used to diagnose multiple myeloma
Craniopharyngeoma
Calcified, intracranial tumors that occur in the supresellar region.
Presenting symptoms include bitemporal hemianopsia and pituitary hormonal deficiencies (e.g., diabetes insipidus, growth hormone deficiency)
Empty sella syndrome
Sella turcica is often enlarged and contains no discernible pituitary gland.
Patient may be asymptomatic or present with hypopituitarism.
Key features of craniopharyngioma
Iron studies in microcytic anemia
- Cause
- MCV
- Iron
- TIBC
- Ferritin
- Transferrin saturation (Iron/TIBC)
Leukemoid reaction versus Chronic myeloid leukemia
- Leukocyte count
- Cause
- LAP score
- Neutrophil precursors
- Absolute basophilia
LAP: Leukocyte alkaline phosphatase
CML is characterized by a low LAP score due to cytochemically abnormal neutrophils
Anticoagulants and their mechanism of action
Treatment of acute deep venous thrombosis/pulmonary embolism
- Mechanism of action
- Therapeutic onset
- Overlap needed?
- Laboratory monitoring?
Skin necrosis at injection site
Classic for heparin induced thrombocytopenia (HIT)
Clinical Manifestations of Fanconi Anemia
- Bone Marrow
- Appearance
- Skin
- Eyes/ears
Fanconi’s anemia caused by defective DNA repaire genes
Diagnosis made by chromosomal breaks on genetic analysis
Acquired causes of Aplastic Anemia
Cancers associated with pernicious anemia
Gastric cancer
Gastric carcinoid tumors
Thalassemias (alpha, beta)
- Disorder (genotype)
- Hb electrophoresis
- Anemia severity
Iron deficiency anemia, alpha thalassemia minor, beta thalassemia minor
- MCV
- RDW
- RBCs
- Peripheral smear
- Serum iron studies
6. Response to iron supplementation
- Hemoglobin electrophoresis
Differential diagnosis of a mediastinal mass
4 Ts
Thymoma
Teratoma (and other germ cell tumors)
Thyroid neoplasm
Terrible lymphoma
Nonseminomatous germ cell tumors
Elevated AFP
May have an elevated beta-hCG
Hairy cell leukemia
Hairy cell leukemia
- Features
- Manifestations
- Diagnosis
- Treatment
Lymphoblasts
Lymphoblasts on peripheral blood smear is a sign of acute lymphoblastic leukemia
Primarily seen in children
Reed Sternberg cells
Characteristic of Hodgkin lymphoma (B cell neoplasm)
Manifests with a mediastinal mass and a painless, enlarged lymph node
Diamond-Blackfan anemia (DBA)
Congenital macrocytic pure red cell aplasia
Presents in the first 3 months of life with pallor and poor feeding.
CBC reveals a normocytic or macrocytic anemia with reticulocytopenia.
Normal WBC and platelet counts.
Short stature, webbed neck, cleft lip, shielded crest, and triphalangeal thumbs
Schistocytes
Seen in microangiopathic hemolytic anemia (e.g., HUS)
Dactylitis (hand-foot syndrome)
Can be the earliest manifestation of vaso-occlusion in sickle cell disease
Presents at 6 months to 4 years with an acute onset of pain and symmetric swelling of the hands and feet.
Differential diagnosis of bone pain in sickle cell disease
- Cause
- Clinical features
Recommendations for lung cancer screening
- Recommended test
- Recommended interval
- Age for screening
- Eligibility for screening based on smoking history
- Termination of screening
Nontender, solitary node in the head and neck
Concerning for squamous cell carcinoma
Indications for specialized RBC treatments
During blood storage, small amounts of leukocyte debris remains in red cell concentrate. These leukocytes release cytokines, which can cause transient fevers, chillds, and malaise, without hemolysis.
Leukoreduction involves reducing the number of leukocytes, which can prevent febrile nonhemolytic reaction and reduce risk of HLA alloimmunization and CMV transmission.
Chronic myeloid leukemia
BCR-ABL fusion gene
Treat with tyrosine kinase inhibitors
