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.
What is the treatment for Thrombotic Thrombocytopenic Purpura (TTP)?
avoid transfusing platelets if possible–> may worsen clots
Need plasma exchange/plasmapheresis which works to filter circulating antibodies against ADAMTS13
Transfusion of FFP can be helpful if plasmapheresis not immediately available
Diagnosis of Choriocarcinoma
Most aggressive type of germ cell tumor
beta-hCG >1000 IU/L
Diagnosis of Yolk sac tumors
Most common testicular malignancy in prepubertal males
AFP is >100 ng/mL
Diagnosis of Seminoma
Germ cell tumor that is a well-defined hypoechoic mass
Moderate elevation of beta-hCG
Staging for CLL
Rai and Binet staging systems
What is Behçet syndrome?
Recurrent aphthous ulcers, genital ulcerations, and uveitis leading to blindness
Treat acute exacerbations of Behçet syndrome with oral prednisone.
What is Tumor lysis syndrome?
Results when the intercellular contents of tumor cells are released into the serum during chemotherapy producing hyperkalemia, hyperuricemia, hyperphosphatemia, and secondary hypocalcemia.
How to prevent Tumor lysis syndrome?
Prevention:
1) IV hydration
2) Hypouricemic agents such as allopurinol, rasburicase, or febuxostat
Transitioning from insulin drip to subQ
When the patient is ready to transition off the insulin drip for diabetic ketoacidosis (DKA), start subcutaneous insulin BEFORE turning off the insulin drip to prevent rebound DKA.
What is fever of unknown origin?
Fever of unknown origin (FUO) is a term used to describe prolonged fevers of >38.3°C (101°F) with no established diagnosis despite a thorough evaluation. The leading causes of FUO include collagen vascular diseases, infections, and malignancies.
Mechanism of Anterior cruciate ligament tear
Anterior cruciate ligament tears are common among running and jumping athletes. They most commonly occur from a non-contact cutting movement, where the patient experiences a “pop” or giving way episode with immediate pain and rapid swelling. The most common mechanism of injury occurs during a cutting movement with the foot planted and tibia externally rotated with a valgus force across the knee, buckling it inward toward mid-line, forcing the tibia anteriorly.
Mechanism of posterior cruciate ligament tear
PCL tears are usually due to a direct blow to the anterior tibia, forcing the tibia posteriorly and tearing the PCL fibers. The PCL acts as the primary restraint to posterior translation of the tibia. Patients with a PCL tear will present in the same manner as this patient, with deep knee pain, effusion, and decreased range of motion, but the mechanism of injury provides important information as to the structure involved. A positive posterior drawer test provides sufficient evidence to diagnose a PCL tear. This is confirmed with an MRI.
Antibiotics in neutropenic fever
All patients with neutropenic fever should receive immediate treatment with an antibiotic that is broad spectrum and covers gram-positive and gram-negative organisms including Pseudomonas. Such antibiotics include piperacillin-tazobactam, meropenem, imipenem-cilastatin, and cefepime.
Granulomatosis with polyangiitis (Wegener disease)
Common vasculitis that presents with upper respiratory lesions, alveolar hemorrhage, glomerulonephritis
Cytoplasmic antineutrophil cytoplasmic antibody (C-ANCA) and antiproteinase-3 antibody.
Sturge-Weber syndrome
Congenital vascular disorder affecting the capillaries. Affects the young
Port-wine stain in the distribution of the trigeminal nerve.
Common complications include seizure, intellectual disability, and glaucoma.
Polyarteritis nodosa
p-ANCA
Positive hepatitis B surface antigen or antibody
Mononeuropathies such as a foot drop.
Goodpasture syndrome
Pulmonary-renal syndrome
Antiglomerular basement membrane antibody
Typically does not involve the upper respiratory symptoms
Churg-Strauss syndrome
Positive peripheral cytoplasmic antibody (p-ANCA)
History of asthma
Eosinophilia
Treatments and cure for paroxysmal nocturnal hemoglobinuria (PNH)
Treatment: Prednisone and Eculizumab
Cure: Allogeneic hematopoietic stem cell transplantation
Peutz-Jeghers syndrome
Autosomal dominant mutation in a tumor suppressor gene (STK11)
hamartomatous polyps of the gastrointestinal tract; mucocutaneous hyperpigmentation of the mouth
Patients of Peutz-Jeghers syndrome are at increased risk of what cancers?
Start screenings at an early age
Gastrointestinal: Colorectal (39%) Gastric (29%) Small bowel (13%) Pancreatic (11%-36%)
Extraintestinal: Breast (32%-54%) Ovary (21%) Cervix (10%) Sertoli cell tumor of the testis (9%) Lung Thyroid
Key words and diagnosis
Severe aplasia- sickle cell aplastic crisis
Blast cells with Auer rods- acute promyelocytic anemia
“Dry tap”- primary myelofibrosis
Reed sternberg cells- Hodgkins Lymphoma
Immune thrombocytopenic purpura (ITP)
Disease of peripheral platelet destruction by circulating autoantibodies
Look for pt presenting weeks after viral infection with nonblanching rash
Stable pt- corticosteroids and IVIG
Unstable- Platelet transfusion
Refractory pts- splenectomy
Hepatic Adenoma
Encapsulated, well circumscribed mass with central necrosis and calcifications on a computed tomography (CT) scan
commonly found in Young, childbearing aged women
Associated with OCPs and anabolic steroids
Treatment for Hepatic adenoma
Hepatic adenomas are benign epithelial tumors that have a risk of malignant transformation and rupture.
Patients should stop taking oral contraceptives, and large or symptomatic tumors should be surgically resected.
Von Willebrand disease (VWD)
Most common inherited bleeding disorder, is an autosomal dominant condition
Low levels of factor VIII because vWF carries factor VIII, and without vWF, factor VIII is easily degraded.
Nosebleeds, oral bleeding, menorrhagia, easy bruising, or postincisional bleeding.
Diagnosis of Von Willebrand disease (VWD)
Ristocetin cofactor assay of the patient’s plasma can measure the capacity of vWF to agglutinate platelets and is used in the diagnosis of VWD
Partial thromboplastin time will be prolonged due to decreased factor VIII
Epididymitis
Testicular swelling in sexually active males
Positive Prehn’s sign (relief of pain with testicular elevation)
Normal lie and cremasteric reflex
Organisms that cause Epididymitis
Younger than 35 years: Chlamydia trachomatis Doxycycline AND IM ceftriaxone
Older than 35 years Escherichia coli Levofloxacin OR ofloxacin
Hemophilia A
X-linked recessive disorder
Factor VIII deficiency
Intrinsic pathway of the coagulation cascade will be dysfunctional (PTT increased)
Everything else normal
community-acquired pneumonia (CAP) treatment
Combination therapy and usually consists of ceftriaxone plus doxycycline or azithromycin
Osteitis fibrosa cystica
Skeletal disorder caused by a surplus of parathyroid hormone (PTH) from overactive parathyroid gland
Can cause lytic lesions on bones
looks like brown tumor with gaint cells
Acute Promyelocytic Leukemia
Subclass of AML
• Arrow pointing to classic Auer rod
• Translocation t(15;17)
• Associated with disseminated intravascular coagulation
• Treatment: Emergent all-trans retinoic acid
Burkitt Lymphoma
• Classic “starry-sky” appearance with sheets
of atypical lymphoid cells (sky) with evenly
dispersed normal macrophages (stars)
• Translocation t(8;14) c-myc
• Jaw or abdomen tumors
• Aggressive non-Hodgkin lymphoma
Hairy Cell Leukemia
- Rare B cell lymphoma (< 2%)
- Tartrate-resistant acid phosphatase stain positive
- Dry bone marrow aspiration
- Hair-like protrusions from cell
Hodgkin Lymphoma
• Reed Sternberg cells, a giant cell with a
multinucleated or bilobed nucleus resembling
owl’s eyes
• Pain with alcohol ingestion
• Cyclical fevers
Chronic Lymphocytic Leukemia
• Smudge cell lacking identifiable cytoplasm
or nuclear structure
• Indolent leukemia, often found incidentally
• Affects older individuals
laboratory findings for von Willebrand disease
Increased PTT
Normal PT
Normal Platelet count
laboratory findings for Hemophilia A`
Increased PTT
Normal PT
Normal platelet count
laboratory findings for DIC
Increased PT, PTT
Increased fibrin split products,
Decreased Platelet count and fibrinogen
Causes of Death
Less than 1- Congenital problems
Adolescents- 1) Accidents. 2) suicide
Adults 45-65 1) Cancer 2) Heart Dz
Adults 65 and up 1) Heart dz 2) Cancer
Waldenstrom macroglobulinemia
IgM
Hyperviscosity syndrome that typically manifests as lethargy, weight loss, Reynaud syndrome, and blurry vision
Tx: Plasmapheresis
Multiple myeloma
CRAB (calcium [elevated)], renal failure, anemia, bone lesions) and IgG/IgA
Monoclonal gammopathy of undetermined significance
IgG/IgA without CRAB
