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 20-40 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

80-90 % 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