Hematology Oncology Board Review II Anas Sawas

Multiple Myeloma Lymphoma Leukemia

Multiple Myeloma  Monoclonal immunoglobulin produced by a single clone of neoplastic plasma cells  Presentation: Elderly patient, chronic back pain, anemia, renal disease, hypercalcemia  Diagnosis: SPEP & UPEP, Bence Jones Protein, bone marrow biopsy, (Rouleaxu formation) skeletal survey and ESR  Treatment: IV hydration, address hypercalcemia and renal failure, pain control, skeletal survey and contact hematology oncology

Lymphoma  Cancer of lymphocytes and present as solid tumor  Hodgkin’s and Non-Hodgkin’s Lymphoma (NHL)  B Symptoms (if present, more aggressive):  Fever  Night Sweats  Weight Loss  Diagnosis: lymph node biopsy

Hodgkin’s Lymphoma  Less common than NHL  Male, bimodal age distribution (age and >55), strong family history, relation to viral infection  Rapid treatment associated with high cure rates  Diagnosis: B symptoms + Local spread to contiguous lymph node (different from NHL)  Pathology: Reed‐Sternberg cell on biopsy

Non-Hodgkin’s  Follicular Lymphoma (40% of lymphomas in adults)  Indolent and often wide spread at presentation  Not curable, but slow growing  Who: older adults  Can transform to aggressive form with high mortality (patient has B symptoms)  Diffuse Large B Cell Lymphoma (40-50% of lymphomas in adults)  Aggressive is commonly symptomatic  About half are curable  Rapidly spreads outside of lymph nodes  Who: all ages, more common in older adults

Leukemia  Neoplastic proliferation of hematopoietic or lymphoid cells cancer of the blood  Diagnosis: bone marrow aspiration  Types:  Lymphocytic vs Myelogenous  Acute vs Chronic  Lymphocytic: cancer of cells that become lymphocytes (B or T cells)  Myelogenous: cancer of cells that become RBCs and PLTs  Acute: CHILDREN; rapid increase in blasts → crowds out other cells in marrow → blasts spill into blood  Chronic: ELDERLY; slow onset, progress over yrs; mature abnormal WBCs

Leukostasis  True Emergency  WBC > 100K, usually AML or CML in blast crisis  Viscous blood plugs circulation  Symptoms: severe hypoxia, headache, dizziness, visual changes, AMS  Treatment:  Induction chemotherapy  Temporize with leukapheresis  Allopurinol (prevent Tumor lysis syndrom), hydroxyurea

Tumor lysis Syndrome (TLS)  Death of many CA cells at once → massive release of intracellular contents → metabolic derangements → final pathway is renal failure  Who: aggressive hematologic malignancies, post induction therapy, radiation therapy and high sensitivity to treatment  Symptoms: nonspecific and related to the electrolyte issue  Diagnosis: Uric acid level, hyperkalemia, hyperphosphatemia, hypocalcemia  Treatment:  Aggressive fluid resuscitation  Treat electrolyte abnormalities  Allopurinol (Slow acting), Rasbuicase (Rapid acting), Phosphate binders (Aluminum Hydroxide), Dialysis  Cardiac monitoring  Repeat labs

Platelet Disorders  Idiopathic Thrombocytopenic Purpura (ITP)  Thrombotic Thrombocytopenic Purpura (TTP)  Hemolytic Uremic Syndrome (HUS)  Disseminated Intravascular Coagulation (DIC)  Heparin Induced Thrombocytopenia (HIT)

Idiopathic Thrombocytopenic Purpura (ITP)  Isolated thrombocytopenia, other labs normal; patient well appearing  Childhood form: acute, self limiting, post immunization or infection  Adult form: insidious, chronic, no preceding illness  Pathophysiology: impaired platelet production; T cell mediated destruction, B cell clone antibodies, splenic clearance of IgG coated platelets  Treatment:  Corticosteroids  IVIG: works fast but short lived $$$  Platelets: temporary hemostatic support; will be destroyed; use in critically ill patients  Splenectomy  RhoGAM: induces mild hemolysis in Rh+ patients → decreases macrophage activity, spares IgG coated platelets from splenic destruction  Chemotherapeutic agents: Rituximab

Thrombotic Thrombocytopenic Purpura (TTP)  Pathophysiology: long platelet chains clog blood vessels and prevent RBC passage  “The Evil Pentad”: low platelets, anemia, fever, acute renal failure, neuro symptoms  Consider this diagnosis if patient has thrombocytopenia, microangiopathic hemolytic anemia (MAHA), +/CNS, no other obvious cause  Risk factors: obese, African American, female, HIV, SLE, drugs (Quinine, Clopidogrel, Ticlopidine)  Laboratory Abnormalities: thrombocytopenia, anemia, ↑ unconjugated bilirubin, ↑ LDH, normal fibrin/fibrinogen, ADAMTS13 tests, vWF gel electrophoresis, DNA analysis; hematuria  Treatment  Give FFP, PET, immune suppressants, splenectomy, IVIG, antiplatelet agents  NO Platelets

Hemolytic Uremic Syndrome  Childhood  Triad: MAHA, thrombocytopenia, acute renal failure  % caused by E. Coli 0157:H7 (Shiga like toxin)  Pathophysiology: Endothelial damage → Thrombin generation promoted → Fibrin deposition → Platelet Fibrin clots  Risk Factors: history of raw hamburger, petting zoo, long term care facility  Diagnosis: presumptive; stool and urine for shiga toxin  Lab Findings: schistocytes, thrombocytopenia, UC bili and LDH, normal fibrin/fibrinogen, negative direct coombs, negative blood cultures for E. Coli  Treatment: mainly supportive Admission for IVFs, PRBC for HgB <6 or unstable VS, HD for anuria, PET for severe cases  No platelets  Antibiotics can increase toxin release

Disseminated Intravascular Coagulation  Characterized by widespread microvascular thrombosis  Consumption of clotting factors and platelets (common)  Decreased blood flow to vital organs → organ failure (rare)  Pathophysiology: Massive inflammation → endothelial damage → cytokine release (TNF, IL ‐6) → impaired anticoagulation & consumption of clotting factors  Lab Findings: ↓ Platelets, ↑ D‐Dimer, ↑ PT/INR, ↓ Fibrinogen  Treatment  Identify and treat underlying cause  Give platelets if <10‐20K or if risk of bleeding and <50K

A 23-year-old with ALL presents with generalized weakness and muscle cramps. The patient states that he started chemo 3 days ago. A basic metabolic panel shows serum potassium of 6.5 mEq/L, calcium of 6.3 mg/dl and a creatinine of 11.1 mg/dL. What management is indicated?  A. Aggressive fluids and admit  B. Aggressive fluids and emergent dialysis  C. Kayexalate and admit  D. Urine alkalinization

A 23-year-old with ALL presents with generalized weakness and muscle cramps. The patient states that he started chemo 3 days ago. A basic metabolic panel shows serum potassium of 6.5 mEq/L, calcium of 6.3 mg/dl and a creatinine of 11.1 mg/dL. What management is indicated?  A. Aggressive fluids and admit  B. Aggressive fluids and emergent dialysis  C. Kayexalate and admit  D. Urine alkalinization

A 23-year-old man with sickle cell disease presents with chest pain, cough and fever. His vitals are HR 132, RR 28, BP 110/65, and T 101.1°F. His chest X-ray shows a right lower lobe infiltrate. Hemoglobin is 8 g/dL. What first line management is indicated?  A. Broad spectrum antibiotics and ICU admission  B. Exchange transfusion  C. Oral antibiotics and follow up with hematology  D. Splenectomy and broad-spectrum antibiotics

A 23-year-old man with sickle cell disease presents with chest pain, cough and fever. His vitals are HR 132, RR 28, BP 110/65, and T 101.1°F. His chest X-ray shows a right lower lobe infiltrate. Hemoglobin is 8 g/dL. What first line management is indicated?  A. Broad spectrum antibiotics and ICU admission  B. Exchange transfusion  C. Oral antibiotics and follow up with hematology  D. Splenectomy and broad-spectrum antibiotics

A 72-year-old man presents with lower back pain and significant weakness. Laboratory analysis is notable for a creatinine of 1.9 mg/dL, calcium of 10.7 mg/dL and total protein of 9.2 gm/dL. Which of the following might you expect to find?  A. Hyperreflexia  B. Lytic lesions on plain film  C. Polycythemia  D. Urinary retention

A 72-year-old man presents with lower back pain and significant weakness. Laboratory analysis is notable for a creatinine of 1.9 mg/dL, calcium of 10.7 mg/dL and total protein of 9.2 gm/dL. Which of the following might you expect to find?  A. Hyperreflexia  B. Lytic lesions on plain film  C. Polycythemia  D. Urinary retention

A 24-year-old man with HIV was recently started on Bactrim. He complains of two days of worsening fatigue and dyspnea. The patient appears pale with scleral icterus. Laboratory testing shows a marked anemia and elevated total and indirect bilirubin. What is the most likely diagnosis?  A. Crigler-Najjar syndrome  B. Gilbert’s syndrome  C. Glucose-6-phosphate dehydrogenase deficiency  D. Immune reconstitution syndrome

A 24-year-old man with HIV was recently started on Bactrim. He complains of two days of worsening fatigue and dyspnea. The patient appears pale with scleral icterus. Laboratory testing shows a marked anemia and elevated total and indirect bilirubin. What is the most likely diagnosis?  A. Crigler-Najjar syndrome  B. Gilbert’s syndrome  C. Glucose-6-phosphate dehydrogenase deficiency  D. Immune reconstitution syndrome

A 52-year-old man with a recent diagnosis of hypertension complains of intense itching after taking hot showers. He also reports increasing fatigue over the prior four months. His review of systems is otherwise normal and takes propranolol for his hypertension. What physical exam finding is most consistent with his diagnosis?  A. Pallor  B. Petechiae  C. Plethora  D. Purpura

A 52-year-old man with a recent diagnosis of hypertension complains of intense itching after taking hot showers. He also reports increasing fatigue over the prior four months. His review of systems is otherwise normal and takes propranolol for his hypertension. What physical exam finding is most consistent with his diagnosis?  A. Pallor  B. Petechiae  C. Plethora  D. Purpura

A 43-year-old woman with breast cancer on chemotherapy presents with a fever to 102°F. She also complains of a cough and generalized fatigue. Physical examination and chest X-ray are unremarkable except for the presence of a mediport. Complete blood count reveals a white blood count of 600 with 30% neutrophils and no band forms. What management is indicated?  A. Administer filgrastim and discharge home  B. Draw blood cultures and await results for treatment  C. Send blood and urine cultures and start Vancomycin and Cefepime  D. Start levofloxacin and admit for pneumonia

A 43-year-old woman with breast cancer on chemotherapy presents with a fever to 102°F. She also complains of a cough and generalized fatigue. Physical examination and chest X-ray are unremarkable except for the presence of a mediport. Complete blood count reveals a white blood count of 600 with 30% neutrophils and no band forms. What management is indicated?  A. Administer filgrastim and discharge home  B. Draw blood cultures and await results for treatment  C. Send blood and urine cultures and start Vancomycin and Cefepime  D. Start levofloxacin and admit for pneumonia

A 32-year-old woman, who is at 20 weeks gestational age, presents to the ED after a seizure. Her vital signs are BP 115/70, HR 105, RR 16, T 37.5°C, and pulse oximetry 98% on room air. On exam, you note some confusion, but otherwise there are no focal deficits. Lab results reveal a hemoglobin of 7 g/dL and platelets of 12,000/µL. A peripheral blood smear reveals schistocytes. Which of the following is the most appropriate treatment for her condition?  A. Delivery of fetus  B. Magnesium sulfate  C. Plasmapharesis  D. Platelet transfusion

A 32-year-old woman, who is at 20 weeks gestational age, presents to the ED after a seizure. Her vital signs are BP 115/70, HR 105, RR 16, T 37.5°C, and pulse oximetry 98% on room air. On exam, you note some confusion, but otherwise there are no focal deficits. Lab results reveal a hemoglobin of 7 g/dL and platelets of 12,000/µL. A peripheral blood smear reveals schistocytes. Which of the following is the most appropriate treatment for her condition?  A. Delivery of fetus  B. Magnesium sulfate  C. Plasmapharesis  D. Platelet transfusion

Which of the following tests is helpful in the diagnosis for iron-deficiency anemia?  A. Basophilic stippling  B. Decreased serum haptoglobin  C. Mean corpuscular volume >100 fL  D. Serum ferritin <15 mcg/L

Which of the following tests is helpful in the diagnosis for iron-deficiency anemia?  A. Basophilic stippling  B. Decreased serum haptoglobin  C. Mean corpuscular volume >100 fL  D. Serum ferritin <15 mcg/L