Hematology Flashcards
Anemia is defined as __________ or ___________
Low hemoglobin
Low hematocrit
Symptoms of anemia are a result of ____________
Decreased oxygen delivery to tissues
Core symptoms of all types of anemia
Fatigue Dyspnea on exertion Palpitations If severe: Syncope and chest pain
Types of macrocytic anemia (MCV > 100)
B12 Deficiency Folate Deficiency Alcoholism Hypothyroidism Liver disease
Types of microcytic anemia (MCV < 80)
Iron deficiency
Thalassemia
Anemia of chronic dz
Sideroblastic Anemia
Types of normocytic anemia (MCV 80-100)
Blood loss
Hemolysis
Anemia of chronic dz
How does a pt with vitamin B12 deficiency present?
Neurological symptoms
Peripheral neuropathy most common
Lab values for vitamin B12 deficiency
Low B12 level
High MMA
May also see: elevated iron, indirect bilirubin, and LDH
Vitamin B12 deficiency can be secondary to medication, such as:
Metformin
PPIs
Cimetidine
Treatment for vitamin B12 deficiency
Vitamin B12 replacement
IM preferred if severe neurological problems
Treatment for folate deficiency
Folic acid
A patient with iron deficiency will present with:
Blood loss
GI or menses
Lab values for iron deficiency
Low serum iron Low ferritin High iron binding capacity High RDW Elevated platelet count
Treatment for iron deficiency
Always look for a cause!
Ferrous sulfate
Complications of ferrous sulfate
Can turn stools black but will have negative guaiac stools
Thalassemia is characterized by hemoglobin having reduced __________
Alpha or beta chains
Thalassemia will have ___________ or ______________ on peripheral smeare
Target cells
Tear-drop shaped cells
Beta thalassemia is diagnosed with _____________
Electrophoresis
Treatment for beta thalassemia
Transfusions
Iron chelation
Lab values for anemia of chronic disease
Low serum iron, low iron binding capacity, normal ferritin
Sideroblastic anemia may be due to (4):
- alcohol
- INH (isoniazid)
- copper deficiency
- zinc toxicity
Most common cause of hemolysis normocytic anemia
sickle cell disease
Ultimate complications of anemia
Arrhythmias
MI
CHF
Malignancy of lymphoid stem cells in bone marrow, migrating to the lymph nodes, spleen, liver and other organs
Acute Lymphocytic Leukemia (ALL)
Most common childhood malignancy, peaking between ages 3-7 y/o
Acute lymphocytic leukemia (ALL)
Children with ____________ have increased rates of ALL
Down syndrome
Signs/Symptoms of ALL:
- Pancytopenia symptoms: fever, fatigue, lethargy. Bone pain
- CNS symptoms: HA, neck stiffness, visual changes, vomiting
Physical exam with ALL:
Pallor, fatigue, petechiae, bruising, hepatosplenomegaly, lymphadenopathy, +/- mediastinal mass
Diagnosis of ALL
Bone marrow - hypercellular with > 20% blasts, anemia, thrombocytopenia
Management of ALL
Oral chemo (>90% remission) Stem cell transplant if relapse
B cell clonal malignancy
Chronic lymphocytic leukemia (CLL)
Most common leukemia in adults overall
chronic lymphocytic leukemia (CLL)
Signs/Symptoms of CLL
Most are asymptomatic (find leukocytosis incidentally)
Fatigue, DOE, increased infxns
Physical exam with CLL
Painful lymphadenopathy
Hepatosplenomegaly
Diagnosis of CLL
- Peripheral smear - lymphocytes with smudge cells (fragile B cells)
- Pancytopenia - thrombocytopenia, anemia
Management of CLL
Observation if indolent
Chemo if symptomatic or progressive
Allogeneic stem cell transplant is curative. Poorer prognosis.
Most common acute form of leukemia in adults
Acute myelogenous leukemia (AML)
Signs/Symptoms of AML
Pancytopenia - anemia, thrombocytopenia, neutropenia
Splenomegaly
Gingival hyperplasia
Bone pain
Diagnosis of AML
Leukostasis (WBC > 100,000)
Bone marrow - Auer Rods
Auer rods on bone marrow
AML
Management of AML
Combo chemotherapy
+/- allogeneic bone marrow transplant after remission
Tumor Lysis Syndrome
Seen with AML
Occurs after chemo induction
Treatment: allopurinol, IV fluids
Granulocyte proliferation seen with well differentiated WBCs
Chronic myelogenous leukemia (CML)
Signs/symptoms of CML
Most asymptomatic until they develop blastic crisis, splenomegaly
Diagnosis of CML
- Cytogenetics or FISH - philadelphia chromosome
2. Strikingly increased WBC counts (> 100,000)
Philadelphia chromosome
CML
Philadelphia CreaM cheese
Management of CML
Chemo
Allogeneic stem cell transplant in severe dz or if failed chemo
Pentad of thrombotic thrombocytopenic purpura (TTP)
- Thrombocytopenia
- Microangiopathic hemolytic anemia
- Kidney failure/uremia
- Neurologic sx - HA, CVA, AMS
- Fever (rare)
Thrombocytopenia sx
Petechiae Bruising Purpura Mucocutaneous bleeding involving the skin, oral, GI, GU Epistaxis
Microangiopathic hemolytic anemia sx
Anemia
Jaundice
Fragmented RBCs/schistocytes on peripheral smear
Primary TTP is due to
autoimmune (idiopathic)
Secondary TTP may be due to:
Malignancy Marrow transplant SLE Estrogen Pregnancy HIV
What is decreased with TTP?
ADAMTS13
Diagnosis of TTP
- Labs - thrombocytopenia, normal coags
- Hemolytic anemia - increased reticulocytes, schistocytes
Coombs negative
Splenomegaly
Management of TTP
- Plasmapheresis - tx of choice - adds ADAMTS13
2. Immunosuppression - corticosteroids, cyclophosphamide
Most common inherited bleeding disorder (autosomal dominant)
Von Willebrand Disease
Characterized as a defect or deficiency in a factor that helps platelets stick to sites of endothelial injury
Von willebrand disease
Signs/Symptoms of von willebrand disease
- bleeding!
Gingival bleeding, epistaxis, menorrhagia and skin bruising
NSAIDs/Aspirin may precipitate an episode
Diagnosis of von willebrand disease
Normal platelet count, normal PT
Von willebrand factor antigen - decreased
Von willebrand factor activity - decreased
Factor VIII activity - decreased
If all abnormal, perform RIPA
First line treatment for von willebrand disease
Desmopressin
Second line: von willebrand factor concentrate
Group of inherited (x-linked recessive [male]) bleeding disorders
Hemophilia
Factor VIII Deficiency
Hemophilia A
Factor IX Deficiency
Hemophilia B or Christmas disease
Signs/Symptoms of hemophilia
Pt who is bleeding into joints, muscles, or GI tract
First episode can occur anywhere from first few months of life up until age 4
Diagnosis of hemophilia
Normal platelet count, normal PT, prolonged aPTT
When pts plasma combined with normal plasma, aPTT will correct itself
Confirmation done by ordering specific assay
Treatment of hemophilia
Hemophilia A - Desmopressin
Hemophilia B - specific clotting factor
Factor XI Deficiency - no prophylactic therapy needed
Most common cause of inherited thrombophilias
Factor V Leiden
Mutant form of coagulation factor that is insensitive to activated protein C (anticoagulant)
Factor V Leiden
Screening in asymptomatic pts should only be done if there are multiple ______________ with DVT formation under age of _______
First degree relatives
50
Otherwise, routine screening not recommended
Lymphoma that peaks in 20s and then in 50s
Hodgkin’s Disease Lymphoma
Reed sternberg cells pathognomonic - B cell proliferation with bilobed or multilobular nucleus “owl eye”
Hodgkin’s Disease Lymphoma
Lymph node involvement with Hodgkin’s Disease lymphoma
Upper lymph node involvement
Neck, axilla, shoulder, chest (mediastinum)
With Hodgkin’s disease lymphoma, sometimes you will see pts with painful lymph nodes with _______________
EtOH ingestion
Although usually pts present with painless lymph nodes
Associated symptoms with Hodgkin’s Disease Lymphoma
fever
weight loss
anorexia
night sweats
Pel Ebstein Fever
Intermittent cyclical fevers x 1-2 weeks
Hodgkin’s disease lymphoma
40% of hodgkin’s disease lymphoma is associated with
Epstein-Barr Virus
Hodgkin’s disease lymphoma has an __________ 5 year cure rate
Excellent
Lymphoma that is most commonly seen after 50 y/o, and has an increased risk with immunosuppression (HIV, viral infxn)
Non Hodgkin’s Lymphoma
Lymphoma with diffuse large B cell that is more common (more aggressive)
Non Hodgkin’s Lymphoma
Waldeyer’s Ring
Tonsils, base of tongue and nasopharynx that are involved in lymph node spread with
Non-Hodgkin’s lymphoma
Lymph node involvement with Non-Hodgkin’s lymphoma
Peripheral multiple node involvement
Axillary, abdominal, pelvic, inguinal, femoral
Extranodal spread seen with non-hodgkin’s lymphoma
GI MC Skin 2nd MC Testes Bone marrow GU Liver, spleen, thyroid, kidney, spine
Survival rate with non-hodgkin’s lymphoma is:
Variable
Acquired myeloproliferative disorder with overproduction of all 3 myeloid cell lines (RBCs, WBCs, platelets)
Polycythemia Vera (Primary Erythrocytosis)
Polycythemia Vera is caused by:
JAK2 mutation
Increased hematocrit in the absence of hypoxia
Primary erythrocytosis
Signs/Symptoms of polycythemia vera
Symptoms due to increased RBC mass
Thrombosis or hyperviscosity
Headache, dizziness, tinnitus, blurred vision, pruritus, fatigue, thrombosis
Pruritus especially after hot bath
Seen with polycythemia vera (primary erythrocytosis)
Episodic burning/throbbing of hands and feet with edema
Erythromelalgia Polycythemia Vera (Primary erythrocytosis)
Physical exam with polycythemia vera (primary erythrocytosis)
Splenomegaly
Facial plethora (flushed face)
Engorged retinal veins
Major Criteria for Polycythemia Vera
- Increased RBC mass, hematocrit and Hgb
- Bone marrow biopsy showing hypercellularity
- JAK2 mutation presence
Minor Criteria for Polycythemia Vera
Decreased serum erythropoietin levels
Criteria for polycythemia vera
All major or first 2 major + minor = polycythemia vera
Management of polycythemia vera
Phlebotomy management of choice
Done until hematocrit < 45% to reduce high risk of venous thrombosis
Low dose aspirin