Heme/Onc Flashcards
Three types of complications of lead poisoning:
- Sideroblastic anemia
- GI (abdominal pain, constipation)
- Neurological (weakness, sensory loss, memory)
Anemia associated with basophilic stippling
Lead poisoning (a sideroblastic microcytic anemia)
What are 2 causes of isolated thrombocytopenia that must be tested for before making a diagnosis of ITP?
HIV and HCV
Classic pentad of TTP
- Thrombocytopenia
- Microangiopathic hemolytic anemia
- Fever
- Renal insufficiency
- Neurologic effects (headache, confusion, coma, stroke)
Treatment for TTP
Emergent plasma exchange
Do NOT transfuse platelets unless there is active severe bleeding, as they will be consumed
Coagulation tests in hemophilia
Prolonged PTT, normal PT
factors 8 and 9 only used in intrinsic pathway, which is measured by PTT
Classic signs of G6PDD on blood smear?
Heinz bodies and bite cells
Classic presentation of hereditary spherocytosis? Diagnostic test?
Presentation: Anemia, jaundice, and splenomegaly (extravascular hemolysis in spleen)
Test: Osmotic fragility test
What problem can be seen in patients with sickle trait?
Hyposthenuria: decreased ability to concentrate the urine, due to sickling in the vasa rectae
(Also seen in sickle cell disease)
What presents with higher O2 saturation on ABG than on pulse-ox?
Methemoglobinemia (hemoglobin oxidized and unable to bind oxygen, so pulse-ox is correctly low, but O2 sat in ABG is calculated based on PO2, which will be normal)
Time course of type 1 and type 2 HIT
Type 1: within 2 days
Type 2: within 5-10 days (but can be sooner if repeat exposure)
Treatment for type 1 HIT? Type 2?
Type 1: No treatment, can continue heparin
Type 2: stop heparin and switch to a novel anticoagulant (don’t switch to warfarin because of its initially prothrombotic effects, and HIT is already a prothrombotic state)
Gold standard test for HIT
Seroronin release assay
Treatment for warfarin-induced skin necrosis
Stop warfarin and give protein C
(Due to rapid decline of protein C when starting warfarin leading to pro-coagulant state, usually in patients with underlying protein C deficiency)
Most common hereditary thrombophilia?
Second most common?
Less common one associated with warfarin-induced skin necrosis?
Most common: Factor V leiden (resistant to inactivation by protein C)
2nd most common: Prothrombin mutations (with increased prothrombin levels)
Associated with warfarin-induced skin necrosis: protein C deficiency
Triad of graft-versus-host disease?
- Maculopapular rash on face, palms, and soles
- Diarrhea with frank or occult blood
- Jaundice and elevated LFTs
(Due to donor T cells attacking host antigens)
Haptoglobin levels in extravascular and intravascular necrosis
Extravascular: may be normal, but may be decreased with rapid hemolysis
Intravascular: decreased
What is one group at risk for anaphylaxis after blood transfusion?
What type of RBC should they get?
IgA deficiency (due to residual IgA in donor products)
These patients should get washed RBCs
What is one group at risk for primary hypotransfusion reaction?
Patients on ACEIs
(Reaction is transient hypotension due to bradykinin that accumulates during blood product preparation, and ACE is normally what breaks down bradykinin)
What transfusion reactions can occur within minutes of transfusion?
Within an hour?
After 1-6 hours?
After 2-14 days?
Minutes: Anaphylaxis or primary hypotension transfusion reaction
Hour: Acute hemolytic reaction
1-6 hours: (FNHTR) (febrile non-hemolytic transfusion reaction) and TRALI (transfusion related lung injury)
1-14 days: delayed hemolytic reaction
Cause of acute hemolytic reaction? Delayed hemolytic reaction?
Acute: existing antibodies, usually against ABO antigens
Delayed: new antibodies after non-ABO antigens (Rh, Kidd, etc)
What should patients with a history of febrile non-hemolytic transfusion reaction receive if they need an RBC transfusion?
Leukoreduced RBCs (washed to reduce as many WBCs as possible)
(FNHTR due to cytokines from WBC in donor blood)
What type of transfusion most commonly leads to TRALI?
Usually platelets or FFP, but can be seen with RBC due to residual plasma
(Due to antibodies in donor plasma against recipient HLA)
What electrolyte abnormality can be see with massive PRBC transfusions?
Hypocalcemia (due to citrate in the RBCs, a calcium chelator)
(Requires e.g. replacement of entire blood volume in 24 hours)
Treatment for chemotherapy-induced nausea
Ondansetron (5HT3 blockers)
Myelodisplastic syndrome
Clonal malignancy of hematopoeitic stem cells take over the marrow and lead to peripheral cytopenias (usually a macrocytic anemia first)
What can myelodisplastic syndrome transform into?
AML
End-organ damage in multiple myeloma
CRAB:
Calcium elevation (due to lytic bone lesions)
Renal insufficiency (light chain/antibodies damage kidney)
Anemia
Bone lesions (non-tender bone pain)
Also at risk for infections (disrupts normal WBCs and antibody production)
Initial test for multiple myeloma
SPEP (serum protein electrophoresis) (will show M-spike)
Also used in Waldenstrom macroglobulinemia and MGUS
Antibodies produced by multiple myeloma? Waldenstrom macrogloblulinemia?
MM: IgG, IgA, or light chain
WM: IgM
Presentation of Waldenstrom macroglobulinemia (3)
- Hyperviscosity syndrome (diplopia, tinnitus, headache, dilated/segmental fundoscopic findings)
- Neuropathy
- Cryoglobulinemia (IgA precipitates in colder parts of body)
B cell leukemia that leads to pancytopenia, splenomegaly, but usually not lymphadenopathy
Hairy cell leukemia
Nature of bone pain in multiple myeloma
Nontender
What are smudge cells classically associated with?
Chronic lymphocytic leukemia (CLL) (Due to abnormally fragile WBCs being damaged during slide preparation)
What that can lead to massive leukocytosis (>50,000)?
Leukemias (ALL, AML, CLL, CML), and leukemoid reaction
Test that can differentiate between chronic myeloid leukemia and leukemoid reaction
Leukocyte alkaline phosphatase (LAP) score: high in leukemoid reaction, low in leukemia
(LAP is a marker of relatively mature WBCs )
Finding on blood smear in Hodgkin lymphoma
Reed-Sternberg cells (“owl’s eyes” due to prominent inclusion-like nucleoli)
Treatment of polycythemia vera
Serial phlebotomy +/- hydroxyurea (use in cases of increased thrombotic risk, like previous thrombosis or advanced age)
Symptoms of polycythemia vera (5)
- Hypertension (due to viscosity)
- Transient visual disturbances
- Erythromyalgia (burning cyanosis in hands and feet)
- Aquagenic pruritis (itching after showers)
- Gouty arthritis (due to increased RBC turnover)
Medications that can lead to folate deficiency (3)
- Phenytoin (decreases absorption)
- Trimethoprim (in Bactrim) (blocks DHFR)
- Methotrexate (also blocks DHFR)
Treatment for paroxysmal nocturnal hemoglobinuria (PNH)
Eculizumab (monoclonal antibody that inhibits complement)
Manifestations of paroxystmal nocturnal hemoglobinuria (PNH) (3)
- Hemolysis leading to hemoglobinuria
- Cytopenias, especially anemia
- Hypercoagulable state leading to thrombosis, especially abrominal (e.g. portal and hepatic vein) and cerebral veins
Diagnostic tests in suspected CLL
CBC+diff, blood smear, and flow cytometry
Lymph node and bone marrow biopsies generally not needed
RBC indices in hereditary spherocytosis
High MCH and low MCV
(Membrane loss and dehydration leads to small, dense RBCs)
(Note that this pattern will not be seen in spherocytosis due to other causes, like G6PDD and autoimmune hemolytic anemia).
Recurrent migratory superficial thrombophlebitis at unusual site: Name? Associations?
Trousseau sign of malignancy a.k.a. migratory thrombophlebitis
Associated with adenocarcinoma, especially of pancreas and lung (also gastric cancer).
Malignancy associated with Auer rods
Acute myeloid leukemia (AML)
Specifically AML with maturation (M2) and acute promyelocytic leukemia (M3)
