Hematology Flashcards
what is the presentation of the patient with ALL
CHILD + Lymphadenopathy + bone pain + bleeding + fever in a CHILD, bone marrow > 20% blasts in bone marrow
a 5 y/o child with lymphadenopathy, bone pain, bleeding, and fever. Bone marrow demonstrates > 20% lymphoblasts
what is the most likely diagnosis
ALL (acute lymphocytic leukemia)
a 60-year-old male complaining of fatigue. Blood tests demonstrate severe anemia, decreased neutrophil count, and small, abnormal B lymphocytes in the bone marrow (>30%) with levels at 90,000 per cubic millimeter. He has painless cervical lymphadenopathy and hepatosplenomegaly.
what is the diagnosis
CLL (chornic lymphcytic leukemia)
what are diagnostic tests for CLL
SMUDGE cells on peripheral smear
mature lymphocytes
a 52-year-old male who reports that he has been feeling very tired lately, and his wife thinks that he looks pale. You order a complete blood count, which shows: Hgb 8.5 g/dL (normal 13.5-17.5); WBC 1,200/microliter (normal 4,500 – 11,000); platelets 70,000/microliter (normal 150,000 – 400,000). The patient is referred for bone marrow biopsy, which shows myeloblasts with Auer rods.
what is the diagnosis
AML (acute myeloid leukemia)
what is the classic presentation of AML on diagnostic testing
blasts + AUer rods in adult patient
a 49-year-old healthy male without complaints, but on a routine complete blood count (CBC) has markedly increased white blood cell count of 40,000 per uL (normal 4500 – 11,000). A peripheral blood smear demonstrates leukocytosis with myeloid cells present at various stages of differentiation, with more mature cells present at a greater percentage than less mature cells. The cytogenetic analysis is positive for the Philadelphia chromosome.
what is the diagnosis
CML (Chronic myeloid leukemia)
what is the lab presentation of anemia of chronic disease
Normal or ↓ MCV, ↓ TIBC, ↑ Ferritin (high iron stores) ↓ serum erythropoietin
what is the treatment of anemia of chronic disease
erythropoietin and tx underling disease
what is the lab presentation of aplastic anemia
The only anemia where all three cell lines are decreased ↓ WBC ↓ RBC ↓ Platelets - will have normal MCV and ↓ Retic
what diagnostic test proves aplastic anemia
Pancytopenia ⇒ decreased WBC, RBC, platelets; most accurate = bone marrow biopsy
what population is commonly affected with folate deficeincy
alcoholics
what is the presentation of G6PD deficiency
After infection or medication (oxidative stress) in an African American male (x-linked) + Heinz Bodies and Bite Cells on a smear (damaged hemoglobin - G6PD protects RBC membrane)
what are triggers for G6PD deficiency
fava beans
antimalarials
sulfonamindes
what diagnostic test for G6PD deficiency
Heinz bodies and bite cells on smear
what physical exam findings are commonly seen with iron deficiency anemia
Pica and nail spooning
African American, pain, family history of blood disorder, Hemoglobin electrophoresis: Hemoglobin S, Blood smear: Sickled RBCs, Howell-Jolly bodies, target cells
what is the diagnosis
sickle cell anemia
what is the treatment of sickle cell anemia
Hydoxyurea
what is seen on blood smear with sickle cell
sickled RBCs, Howell-jolly bodies and target cells
what factor is diminished with Hemphilia A
facotr VIII
what factor is diminished with Hemophilia B
Factor IX
a 30-year-old woman with a recurrent history of nosebleeds and heavy menses. She recently read that taking a baby aspirin was good for the heart. However, ever since she started taking aspirin, she has been experiencing more and more nosebleeds. Her father and paternal uncle similarly have histories of prolonged nosebleeds. Labs show increased PTT, normal PT, and increased bleeding time.
what is the diagnosis
VWD
what is the treatment of VWD
DDAVP (desmopressin)
or tranfusion if massive bleeding
what is virchows triad
blood stasis
hypercoagulable state
vascular injury
what is factor V leiden
procoagulant clotting favor - amplifies production of thrombin a clot formation
how is factor V leiden diagnosed
activated protein C resistance assay
normal PT/PTT
what is the treatment of factor V leiden
LMWH bridge to warfain; long term antithrombotic therapy is not recommended
what is protein C deficiency
vitamin K dependent anticoagulant liver protein that stimulates fibrinolysis and clot lysis (inactivates factor V and VIII) – potentiated by protein S
what is the treatment of protein C deficiency
heparin and oral anticoagulation for life
what is protein S deficiency
vitamin K dependent that is a cofactor for activated protein C, which inactivates procoagulant factors Va and VIIIa ⇒ reducing thrombin generation
what is antithrombn III deficiency present with
recurrent venous thrombosis and PE, repetitive intrauterine fetal death (IUFD)
what is antithrombin III
natural anticoagulant; inhibits thrombin (IIa), Xa and other proteases; potentiated by Heparin
what is antiphospholid antibody syndrome
autoimmune; often associated with SLE; characterized by thromboses and recurrent spontaneous abortions
how is antiphospholipid antibody syndrome diagnosed
lupus anticoagulant
anticardiolipin
DRVVT test
prolonged PTT
what is the treatment of Antiphospholipid antibody syndrome
High dose IV heparin with thrombotic events then oral anticoagulation indefinities
what is the presentation of Hodgkins lymphoma
painless lymphadenopathy + bimodal age distribution (15-35) and (>60)
Fever, chills, and night sweats for > 1 month
Painless enlarged posterior cervical and supraclavicular lymph nodes Virchow’s node
what is the diagnostic test for Hodgkin lymphoma
CXR - mediastinal adenopathy
excisional bx of lymph nodes with Reed-Sternberg cells
what is the presentation of Non-Hodgkin lymphoma
immunocompromised (HIV) patient with GI symptoms and painless peripheral lymphadenopathy
what is polycythemia vera
malignancy of the bone marrow that results in overproduction of RBC and can also affect platelets and WBCs
what are classic symptoms of polycythemia vera
pruritis s/p hot baths
swelling, burning pain and rubor of hands and feet
what is erythromelalgia
swelling, burning pain, and rubor of the hands and feet
what are the 4 H’s of polycythemia
Hypervolemia, Histaminemia, Hyperviscostity and Hyperuricemia
what is the treatment of polycythemia
repeated phlebotomy to lower hematocrit to ≤ 42%
what is idiopathyic thrombocytopenic purpura (ITP)
Autoimmune reaction to platelets usually after a viral illness
what is the treatment of ITP
children - supportive care (IVIG for refractory cases)
adults - prednisone
what are causes of Thrombotic Thrombocytopenia (TTP)
After drugs: Quinidine, cyclosporine & pregnancy
Inhibition of ADAMTS13
what is the presentation of TTP
Adults
Purpura and “FAT RN”- Fever, Anemia, Thrombocytopenia, Renal failure, Neurological symptoms
what is treament of TTP
steroids, plasmapheresis
what is the presentation of HUS
Post-infection: E.coli or Shigella
Children
Severe kidney problems
what is treatment of DIC
cyopercipitate, FFP, platelet transfusion (if <30,000), heparin and treat the cause
