Heme/Onc Flashcards
most common inherited cause of hyper coagulability
Factor V Lediden
presence of kappa or lambda light chain proteins on urine electrophoresis
Multiple Myeloma
Rouleaux formation of RBCs
punched out lesions on Xray
hypercalcemia
Multiple Myeloma
medication that increases fetal Hgb levels, increases water content of RBCs and decreases sickling deformity of RBCs
Hydroxyurea
hemolytic anemia
venous thrombosis of large vessels
pancytopenia
CLASSIC FOR…
Paroxysmal nocturnal hemoglobinuria
Auer Rods seen, think..
AML
What is prolonged in Hemophilia B (Christmas dz)
PTT
Hemophilia B effects factor IX, which is part of intrinsic pathway..therefor PTT prolonged
10 yo African American male put on trimethoprim-sulfamethoxazole twice daily for a UTI. Comes back later with pallor, abdominal pain and back pain. Peripheral smear has SHISTOCYTES and HEINZ BODIES. Dx?
G6PD deficiency
Which drug is the initial management of choice for heparin induce thrombocytopenia?
Argatroban
Common presentation of sickle cell anemia in children under 2?
Dactylitis
C282Y HFE genotype associated with
Hemochromatosis
Thrombotic Thrombocytopenia Purpura is a disorder of ______
Platelets
Platelets are part of the ______ pathway
Primary coagulation
PTT and PT in Thrombotic Thrombocytopenia Purpura
Both normal!!
Decreased ADAMTS13 serum levels found in…
Thrombotic Thrombocytopenia Purpura (TTP)
Positive neurological symptoms
Schistocytes on periph smear
Thrombotic Thrombocytopenia Purpura (TTP)
69 yo male develops HA, fevers, purpuric rash. CSF pos for gm neg bacteria. Few hours later, pt develops spontaneous bleeding @ venipuncture site. Initial labs.. elevated PT, PTT, INR. Also showed decreased fibrinogen levels. What is diagnosis?
disseminated intravascular coagulation (DIC)
Pathological activation of coagulation system, leading to widespread microthombi and a subsequent phase of severe thrombocytopenia
DIC
Associated w thrombosis, widespread hemorrhages
DIC
Common after gm negative bacterial infections
DIC
Can be brought on by malignancy, OB complications, massive tissue injury, rocky mountain spotted fever, liver dz, aortic aneurysm, acute respiratory syndrome
DIC
PT, PTT, INR in ITP and TTP
should all be normal!
Non contiguous, extra nodal spread
Non Hodgkins Lymphoma
Bimodal distribution of increased incidence in 3rd and 6th decades
Reed Sternberg cells
Painful lymph nodes w ETOH ingestion
Hodgkins Lymphoma
Associated w EBV
Burkitts Lymphoma
What happens to serum ferritin levels in Anemia of Chronic Dz
INCREASES!
Philadelphia Chromosome (translocation between chromosome 9 and 22) seen in..
CML
First line therapy for TTP
Plasmapheresis (plasma exchange therapy)
..this removes all excess ADAMTS 13 anitbodies and replaces ADADMTS 13
If AML pt is receiving chemo and develops tumor lysis syndrome as a complication, what drug can be given?
Allopurinol
Most useful diagnostic tool in patients with a negative Coombs test and periph smear showing microcytic, hyper chromic, sphere-shaped cells
Osmotic Fragility Test
Hereditary Spherocytosis
Due to defect in blood cell membrane, leading to sphere-shaped RBCs and increased cell fragility
Hereditary Spherocytosis
Treatment for moderate Type I von Willebrand Disease
Desmopressin
would also use for Hemophilia A
What is commonly seen as a consequence of thrombocytopenia?
Petechiae
What is the most common clinical presentation of pts with sickle cell trait?
Episodic hematuria
may also have isosthenuria, the inability to concentrate urine
Sickle cell trait is normally….
asymptomatic
Hemophilia A lacks…
Factor VIII
Condition of increased arterial and venous thrombi formation as well as frequent miscarriages
Antiphospholipid syndrome
34 yo female has an increased PTT. a mixing study is performed and there is no correction of the increased PTT. what does she have
Antiphospholipid syndrome
Chemotherapeutic agent that can also be used intrapleurally for pleurodesis
Bleomycin
What organism is most commonly associated with the development of Hemolytic Uremic Syndrome (HUS)
Enterohemorrhagic E. Coli 0157:H7
Shigella and Salmonella can also cause
Hemophilia B pt sprains ankle (grade 3), what is routine management of this patient?
Factor IX concentrates
JAK2 mutation is associated with
Polycythemia Vera
Headache, dizzniess, tinnitus, blurred vision and increasing pruritus of the last 2 weeks that worsens after hot showers…
Polycythemia Vera (JAK2 mutation)
PE shows flushed face, engorged retinal veins w no evidence of papilledema. Increased hemoglobin and hematocrit
Polycythemia Vera (JAK2 mutation)
What is the treatment of refractory immune/idiopathic thrombocytopenic purpura (ITP)
Splenectomy
…removal of the spleen is the definitive management of the splenic destruction of the platelets
Management of severe thrombocytopenia (platelet count of
Platelet transfusion
Increase in: Basophils Platelets RBCS Hematocrit
Polycythemia Vera
NO INCREASE IN LYMPHOCYTES!!!
First line management of sickle cell crisis?
IV hydration and oxygen. Pain medication is also administered
Long term management to reduce frequency of crisis. Also can be used in SEVERE pain crisis
Hydroxyurea
Positive Coombs test is associated with..
autoimmune hemolytic anemia
Folate deficiency B12 deficiency Alcoholism Liver dz Hypothyroidism
..all cause?
Macrocytic Anemia
In addition to G6PD deficiency, where else can Heinz Bodies be seen in a peripheral smear?
Alpha Thalassemia Intermedia
6 mo year old male is brought into pediatric ER when his mom noticed he was “pale and yellow”. CBC showed severe anemia and hypo chromic microcytosis. A peripheral smear shows: numerous target cells and teardrop cells (dacrocytes). Hgb electrophoresis shows:
Hgb A: decreased
Hgb A2: increased
Hgb F: increased (90%)
What does he have?
Beta Thalassemia Major (Cooley’s Anemia)
In children, this is most commonly seen after a viral infections (such as the flu)
Idiopathic (Immune) Thrombocytopenic Purpura (ITP)
The development of antibodies against platelets with associated splenic destruction of platelets
Idiopathic (Immune) Thrombocytopenic Purpura (ITP)
Management of Idiopathic Thrombocytopenia Purpura (ITP) in children
Observation OR
IV immunoglobulin
First line management of Idiopathic Thrombocytopenia Purpura (ITP) in adults
Corticosteroids
In children, classic presentation is: abdominal pain w constipation varying neurologic symptoms (ie difficulty concentrating) anemia metabolic acidosis
Lead poisoning
CBC showing/Periph smear show:
Anemia
Microcytosis
Basophilic stippling
Lead poisoning
Antibodies against ADAMTS 13 leading to large vonWillebrand multimers, causing platelet activation
Thrombolic Thrombocytopenic Purpura (TTP)
Auto antibodies against glycoprotein IIb/IIIa receptor on platelets commonly following a viral infection
Idiopathic Thrombocytopenic Purpura (ITP)
Mutated Factor V that is resistant to breakdown by activated protein C
Factor V Leiden
Pathologic activation of the coagulation system causing micro thrombi and subsequent thrombocytopenia
Disseminated intravascular coagulation (DIC)
Platelet activation by exotoxins (such as Shigella toxin or Shigella like toxin released from Enterohemorrhagic E. Coli)
Hemolytic Uremic Syndrome (HUS)
Aplastic crisis in sickle cell anemia patients can be precipitated by ____ infections
Parovirus B-19
Ice/clay cravings progressive dysphagia pallor of conjunctiva atrophic glossitis angular chelitis spooning of nails esophageal webs upon endoscopy
Iron deficiency anemia
large B cells with bilobed or multi lobar nuclei giving it an “owl eye” appearance due to eosinophilic inclusions in the nuclei
Reed-Sternberg cells, found in Hodgkins Lymphoma
First line management of warm antibody type autoimmune hemolytic anemia in a patient with SLE?
Corticosteroids
Treatment for cold antibody hemolytic anemia
Avoid the cold
Argatroban is what kind of drug?
direct thrombin inhibitor
First line management of polycythemia vera?
Phlebotomy
First line management of Heparin overdose?
Protamine sulfate
A patient with severe B12 deficiency is being treated with IM B12 supplementation. Which electrolyte abnormality is a common complication of B12 replacement therapy?
HYPOKALEMIA
With IM B12 replacement, hypokalemia may result as new blood cells are being formed, since most of the body’s potassium is found intracellularly.
