Hematology/Oncology Flashcards
AA can be differentiated from a hypoplastic variant of myelodysplastic syndrome (MDS) with decreased bone marrow cellularity by finding dysplastic cells and cytogenetic abnormalities typical for MDS.
Aplastic anemia associated with: auto-immune, treatment for hyperthyroid, gold exposure
Tx: young<50 (stem cell transplant), age>50 ATG, prednisone, cycosporine
MDS is typically hypercellular marrow, dysplastic neutrophils, can cause panctyopenia, has risk of becoming AML
- A group of patients with low-risk MDS with the −5q cytogenetic abnormality could be treated with the immunomodulatory drug lenalidomide if they are transfusion dependent. Lenalidomide has been approved for this indication and is effective in decreasing transfusion requirements.
- Younger patients can get stem cell transplant
Causes of pure red cell aplasia_____________
auto-immune, parvo b19, phenytoin/isoniazid, leukemias
*especially effects patients with underlying disorders (sickle cell), malignancies
The triad of neutropenia, splenomegaly, and rheumatoid arthritis is called ________
Felty syndrome
Tx: G-CSF
CML is a clonal hematopoietic stem cell disorder characterized by translocation of the long arm of chromosomes 9 and 22 [t(9;22), Philadelphia chromosome] leading to _________
BCR-ABL fusion gene
Blast crisis is considered to be secondary AML.
CML, peripheral blood neutrophilia along with immature myeloid forms, such as myelocytes and metamyelocytes
Tx: Tyrosine kinase inhibitors (TKIs) are highly effective and are the treatment of choice for patients with chronic phase CML. Three TKIs, imatinib, dasatinib, and nilotinib, are FDA approved for the initial treatment of CM
TKIs are contraindicated during pregnancy, so either close monitoring of patients off treatment or a switch to interferon is indicated.
Essential thrombocytosis
Patients with platelet counts greater than 1,000,000/µL (1000 × 109/L) are at risk for bleeding and should also be treated with hydroxyurea to lower the platelet count. Other therapies for patients who do not respond to hydroxyurea or who experience significant adverse effects include anagrelide and interferon-α. Low-dose aspirin is considered in all patients at high risk and in patients at low risk who have vasomotor symptoms.
Myelofibrosis
Ruxolitinib is reserved for treatment in patients with debilitating symptoms who are not candidates for hematopoietic stem cell transplantation. Hydroxyurea may be used for patients with low-risk PMF
___________may present with a reduced total leukocyte count and features of disseminated intravascular coagulation.
Acute promyelocytic leukemia
Acute promyelocytic leukemia, characterized by poorly differentiated leukocytes with distinctive primary granules that contribute to coagulopathy (Figure 4) and chromosomal translocation t(15;17),
Both ALL and CML can carry the BCR-ABL phuladelphia chromosome
MGUS: MGUS is characterized by an M protein level less than 3 g/dL (or less than 500 mg/24 h of urinary monoclonal FLCs), clonal plasma cells comprising less than 10% of the bone marrow cellularity
MM vs waldenstrom
The clinical presentation is similar to that of multiple myeloma except that (1) organomegaly is common in Waldenström macroglobulinemia and is uncommon in multiple myeloma and (2) lytic bony disease and renal disease are uncommon in Waldenström macroglobulinemia but are common in multiple myeloma.
Neuropathy
Patients with Waldenström macroglobulinemia can develop a hyperviscosity syndrome, with symptoms including altered mental status and diverse central nervous system symptoms, dilated retinal veins on funduscopic examination, and mucosal bleeding from impaired platelet function and dysfibrinogenemia.
Lenalidomide (MM, MDS subtype) and pomalidomide carry a risk of venous thromboembolism (VTE), and patients are considered for thromboprophylaxis.
Bortezomib is associated with a high risk of herpes zoster reactivation; prophylactic therapy with acyclovir is recommended.
All amyloid deposits have a characteristic apple-green birefringence under polarized light microscopy of the tissue with Congo red staining.
Fat pad biopsies are appropriate for amyloid diagnosis
Amyloid associated with MGUS/MM = AL Amyloid
Amyloid associated with kidney injury = B2 microglobin
Patients with immunoglobulin light-chain amyloidosis who are ineligible for autologous hematopoietic stem cell transplantation should receive treatment with melphalan- or bortezomib-based chemotherapy regimens.
Treatment of cryoglobinemia with hyper-viscosity
Patients with neurologic symptoms from the hyperviscosity are treated with emergent plasmapheresis, along with treatment of the underlying plasma cell dyscrasia to prevent production of cryoglobulins
Bite cells characteristic of ____
target cells____
G6PD deficiency
Thallesemia, splenectomy
CKD hemoglobin goal~____
10
An important distinction to make is that folate deficiency leads to an elevation in homocysteine levels with normal levels of methylmalonic acid, whereas cobalamin deficiency has increases in both metabolites.
Because folate is absorbed in the jejunum, small bowel diseases such as amyloidosis, celiac disease, or inflammatory bowel disease can also inhibit folate absorption.
Cobalamin deficiency should be excluded before patients receive folate therapy; although blood abnormalities may improve with folate supplementation, the neurologic complications of cobalamin deficiency would continue to progress.
Iron should be restricted in thalassemia, typically patients have plenty of intracellular stores, risk of iron overload
Thallesemia should only be supplemented with Folate, avoid iron
Hereditary spherocytosis: auto dominant, spectrin mutation, elevated MCHC
Cx: splenomegaly, pigmented gallstones
Folate supplementation needed
Due to spleen complications, need vaccination for Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitides
G6PD (X linked recessive)
-Triggers: Dapsone, sulfa, rasburicase, illness
G6PD evaluation involves a fluorescent spot test used to detect NADPH. A positive result shows lack of fluorescence. Evaluation should not be performed during an acute hemolytic episode
NADPH depletion, less reduction equivalents
Sickle cell disease HgB goal____
10 (Specifically in a pre-op scenario)
Except pregnant females, can avoid transfusion, symptom based transfusion strategy
Exchange transfusion is used for acute stroke or retinal artery occlusion or in patients with severe acute chest syndrome. When indicated, the target hemoglobin S level should be less than 30%.
Drugs associated with hemolytic anemia ______
Dapsone, Cephalosporins, Levofloxacin, Methyldopa, Nitrofurantoin,
Coombs +, spherocytes
Conditions associated with cold antibody hemolytic anemia (IgM)
Infectious (Mycoplasma and Epstein-Barr virus), lymphoproliferative (IgM MGUS, Waldenström macroglobulinemia, other B-cell non-Hodgkin lymphomas)
Tx: Rituximab
*You can treat warm antibody disease with steroids, IVIG, rituxan
MAHA syndromes : Schistocytes, consumption (thrombocytopenia)
Examples of MAHA syndromes: HUS, TTP, DIC
Patients with artificial valves or those with left ventricular assist devices can develop anemia resulting from erythrocyte fragmentation known as macroangiopathic hemolysis
HUS Treatment: Supportive
TTP treatment: Plasmapharesis (ADAMTS13 deficiency due to destruction)
PNH caused by mutation in CD55, 59 that usually protect erythrocyte from complement destruction
Patients lacking these proteins develop episodic hemolysis, marrow aplasia, and thrombosis.
Dx: Flow cytometry
Tx: Eculizumab is associated with Neisseria infections, so patients should receive meningococcal vaccination before use.
Hemachromatosis (Auto dominant):
elevated transferrin saturation greater than 45% and an elevated serum ferritin level; diagnosis is confirmed through genetic testing.
Asymptomatic family members of patients with hemochromatosis and a serum ferritin level less than 300 ng/mL (300 µg/L) (men) or 250 ng/mL (250 µg/L) (women) can be observed
Tx: Phlebotomy
Porphyria cutanea tarda is characterized by cutaneous blisters, often on the hands, and hypertrichosis; it responds well to phlebotomy. (Iron overload condition)
Drug related causes of TTP:
Drugs such as ticlopidine, quinine, cyclosporine, gemcitabine, and vascular endothelial growth factor inhibitors (such as bevacizumab) can cause TTP. Drug abuse with oxymorphone, 3,4-methylenedioxymethamphetamine (“ecstasy”), and cocaine has also been reported to cause TTP.
Hallmark of TTP is neurological decline, kidney injury
Initial treatment involves therapeutic plasma exchange to remove the high-molecular-weight vWF multimers and replace the deficient ADAMTS13–> Rituximab
HUS (Typical vs Atypical)
-Atypical: other factors, not associated with EHEC
severe HUS treatment: Plasma exchange, eculizumab
Conditions associated with ITP
systemic lupus erythematosus, chronic lymphocytic leukemia, HIV, hepatitis C, or Helicobacter pylori infection
Tx: Recommended initial therapy includes a short course (<6 weeks) of prednisone or dexamethasone. The response to intravenous immune globulin (IVIG) is faster and may be indicated in patients with more severe thrombocytopenia and life-threatening bleeding.
Antiplatelet antibody testing is specific but has low sensitivity, so it is not recommended in the diagnosis or management of immune thrombocytopenic purpura.
