Heme/Onc Flashcards
Patient presents with what looks like anemia. What is the next step in diagnosis?
Complete blood count to look at hemoglobin, hematocrit and mean corpuscular volume
what does labs of anemia due to hemolysis look like?
Normocytic anemia (85-100) Elevated bilirubin (normal <1) Elevated LDH (normal<250) Low haptoglobin (normal 50- 150)
What is pernicious anemia?
In pernicious anemia, there is either:
1) antibodies against parietal cells destroy the cells that produce intrinsic factor or
2) there is antibody inactivation of intrinsic factor
Either way this leads to deficiency of IF–> B12 deficiency
What is the Go to test for microcytic anemia?
Ferritin-
Elevated in anemia of chronic disease
Low in iron deficiency anemia
Normal in thalassemia
Thalassemia with normal hemoglobin electrophoresis is…
alpha thalassemia because if alpha is defective and whatever alpha is made is still able to pair with normal beta chain.
Elevated iron in a microcytic anemia means..
Sideroblastic anemia (could be part of developing malignancy) Get bone marrow biopsy to confirm.
Examples of diseases causing normocytic anemia (MCV 85-100)?
1) Hereditary Spherocytosis (hemolytic)
2) Anemia of Acute blood loss
3) Paroxysmal Nocturnal Hemoglobinuria (hemolytic)
4) G6PD Deficiency (hemolytic)
5) Sickle Cell (hemolytic)
What kind of anemia does Paroxysmal nocturnal hemoglobinuria cause?
Normocytic Hemolytic anemia caused by deficiency in the PIGA gene—
GDI anchor fails and RBCs become susceptible to endogenous complement fixation.
Dark urine, usually upon awakening (due to hemosiderin), abdominal pain
Diagnose w/ Flow cytometry looking for deficiency of CD55 & CD59
Labs of a hemolytic anemia will show:
Elevated bilirubin (normal < 1)
Elevated reticulocytes ( Greater than 2%)
Treatment for hereditary spherocytosis?
- Spherocytes on smear
- Positive osmotic fragility test
- Treat mild disease with vitamins ( Folate)
- Severe anemia– splenectomy
Pathophysiology of hereditary spherocytosis cause?
Caused by deficient red blood cell membrane protein such as spectrin, Ankyrin, or band 4.1 which causes weird shaped RBC to get stuck in spleen.
What is the test of choice for a non-tender lymph node?
If you suspect lymphoma- Do excisional biopsy
Four stages of lymphoma:
Stage 1-single node
Stage 2- Multiple nodes on the same side of the diaphragm
Stage 3- Multiple nodes on both sides of the diaphragm
Stage 4- Disseminated disease; marrow or blood
How to diagnose Hodgkins lymphoma?
Excisional biopsy will show Reed sternberg cells (+ for CD15 and CD30)
What is the treatment for Non-Hodgkins lymphoma/ B cell lymphoma)?
Patients w/ stage 2A or better get radiation
Patients w/ stage 2B (B symptoms) or worse get CHOP- R:
cyclophosphamide, vincristine & prednisone w/ rituximab
What is the treatment for Hodgkins lymphoma?
ABVD
Adriamycin/Doxorubicin, Bleomycin, vinblastine, dacarbazine)
What is multiple myeloma?
Plasma cell dysfunction making Immunoglobulins against phantom antigen (useless)
So pt gets Recurrent infections because they have all this useless antibodies
CRABS- Hypercalcemia, Renal failure, Anemia, Bone lytic lesions
How do you diagnose multiple myeloma?
Bone marrow biopsy is required to definitively diagnose MM
Hgb electrophoresis (Spep) shows M spike
Urine electrophoresis (upep) will show bence jones protein
Elevated protein gap
What is the treatment for multiple myeloma?
Everyone gets Chemo- Thalidomide or Bortezomib + Melphalan & prednisone
Age < 70 Also do stem-cell transplant
Age> 70 or no donor then just chemo
What is Monoclonal gammopathy of undetermined significance (MGUS)?
No anemia, no renal failure, no hypercalcemia, or bone pain. Only plasmacytosis of <10% but with (+) M spike.
Also (-) no bence jones protein on Upep
Surveillance q6months with labs to catch possible transformation to MM.
What is Waldenstrom Macroglobulinemia?
Lots of IgM (pentad) production which accumulates and causes 1) Peripheral neuropathy and 2) hyperviscosity syndrome
Elevated M-spike on Spep
How is Waldenstrom Macroglobulinemia diagnosed?
Elevated M-spike on Spep
Bone marrow biopsy > 10% lymphoplasmacytic lymphoma
What is the treatment for Waldenstrom Macroglobulinemia?
Plasmapheresis for hyperviscosity
Chemo w/ Rituximab regimen + haematopoietic stem cell transplant (HSCT)
No treatment if asymp and elderly
What is the treatment for Von willebrand disease?
DDAVP
Desmopressin exerts its haemostatic effect by:
1) Inducing synthesis of the von Willebrand factor (VWF) by endothelial cells.
2) Stimulating release of the VWF from its storage sites in endothelial cells.
3) Cleaving the large VWF multimers circulating in plasma into smaller multimers.
Indications for transfusion of platelets?
<20,000 – to reduce risk of spontaneous hemorrhage
<50,000 AND bleeding
What is the FASTEST way to reverse coumdin/ warfarin effects?
Fresh frozen plasma
What is the treatment for TTP?
Plasmapheresis
How do you treat Heparin induced thrombocytopenia (HIT)?
Stop all heparin products
Use lepirudin to treat
What is Thrombotic Thrombocytopenic Purpura (TTP)?
ADAMTS13 deficiency
Fever Anemia (microangiopathic hemolytic anemia presenting with severe fatigue) Thrombocytopenia Renal Neuro Symptoms
3 or more of this pentad with schistocytes on the blood smear and a normal INR/PTT, they likely have TTP.