Hematology Flashcards
Low ferritin, normal hemoglobin in female athlete with fatigue. Rx?
100 mg elemental iron daily
Enteric coated prep is not sufficient, nor is 50 mg
Iron absorption sites
Duodenum, proximal jejunum
Chronic ITP: + has had splenectomy. Moderate thrombocytopenia: Mx
Observation
Hematologic side-effect of ganciclovir
Neutropenia
Anticoagulation mx for planned minor opthalmologic or dental procedure such as tooth extraction
Continue anticoagulation
Eg:cataracts, BCC or SCC extraction
Very low platelet count in HIV+ patient: Commonest Cx
ITP
ITP Rx not responsive to glucocorticoids
Splenectomy, rituximab
ESRD: target hemoglobin
10 to 11.5
Newly diagnosed HIV with primary HIV thrombocytopenia: Rx
HAART
if no bleeding
Finding in PHAT not seen in ITP
Splenomegaly
HIV-associated thrombocytopenia (previously referred to as “primary HIV-associated thrombocytopenia” [PHAT]) is the most common cause of low platelet counts encountered in HIV-infected patients. Clinically, this condition is similar to primary immune thrombocytopenia (ITP), except that splenomegaly is more commonly noted in patients with HIV. Platelet counts are often higher in HIV-infected patients, and mild thrombocytopenia occasionally resolves without therapy [12-14]. (See “Immune thrombocytopenia (ITP) in adults: Clinical manifestations and diagnosis”.)
The etiology of thrombocytopenia in patients with HIV infection is complex. Bone marrow examination, as in primary ITP, reveals normal or increased numbers of megakaryocytes in the face of reduced numbers of circulating platelets. This combination suggests the presence of ineffective platelet production and/or increased peripheral destruction. Kinetic studies using radiolabeled autologous platelets from HIV-infected individuals have shown that both factors contribute: there is more than a 50 percent reduction in platelet survival and a 50 percent reduction in platelet production [15].
RCA
- Participant interview, chart review.
- Error identification
- Structured analysis of factors:
- Communication structures
- Human resources and leadership
- Environmental
- Information management
- Create an action plan
Pseudothrombocytopenia: PathMex
antibodies against EDTA
0.1% of population
Schistiocytes, MAHA, LDH up, thrombocytopenia
TTP
Monocytosis: causes
- Primary: Acute monoblastic leukemia, Acute myelonmonocytic leukemia
- Secondary:
- TB, brucellosis, listerosis,
- SLE, vasculitis, Inflammatory bowel disease
- Lymphoma
Myelodysplastic syndrome-Myeloproliferative syndrome partly defined by absolute monocyte count > 1000
Myelomonocytic leukemia
Mechanism by which hydroxyurea works in sickle cell
Increases Hb F
only drug in widespread use for stimulating HbF production in patients with SCD It is generally assumed that most of the beneficial effects of hydroxyurea in patients with SCD are due to this effect [20-22].
Meperidine: why not?
metabolized into a compound (normeperidine) that is relatively toxic, and associated with tremulousness, delirium and seizures.
Accumulation of the metabolite is most likely to occur during repeated administration, and dose escalation, and in renal insufficiency.
Most important way to estimate bleeding risk:
History, family Hx
Normal platelet count, normal clotting studies in patient with personal and family history of mucosal bleeding
von Willebrand’s
ristocetin cofactor analysis
initial screening tests for VWD
- Plasma von Willebrand factor (VWF) antigen (VWF:Ag)
- Plasma VWF activity (ristocetin cofactor activity, VWF:RCo and VWF collagen binding VWF:CB)
- Factor VIII activity (FVIII)
- If there is still a high index of suspicion for VWD in the face of normal initial results, further testing is carried out which first includes repeating the initial tests. If the repeat VWF levels are normal, other causes of bleeding should be investigated. If one of the VWF tests is abnormal, the following specialized assays should be performed to determine the type of VWD (table 2):
●VWF multimer distribution using gel electrophoresis
●Ristocetin-induced platelet aggregation (RIPA)
(ristocetin cofactor activity, VWF:RCo and VWF collagen binding VWF:CB)
●Factor VIII activity (FVIII)
If there is still a high index of suspicion for VWD in the face of normal initial results, further testing is carried out which first includes repeating the initial tests. If the repeat VWF levels are normal, other causes of bleeding should be investigated. If one of the VWF tests is abnormal, the following specialized assays should be performed to determine the type of VWD (table 2):
●VWF multimer distribution using gel electrophoresis
●Ristocetin-induced platelet aggregation (RIPA)
DIC+Auer Rods+t(15;17)
Acute promyelocytic leukemia
Hematopoietic neoplasms involving precursor cells committed to giving rise to granulocytic, monocytic, erythroid, or megakaryocytic elements
AML
PML + RAR-α
AML: WHO Classification
- AML with characteristic genetic translocation
- AML with multiple lineages
- AML and MDS, therapy related
- Not otherwise classified
CLL, serious recurrent infections: Rx
IVIG
APL: describe smear
The white cell count in this type of APL is usually low with only a few circulating malignant promyelocytes. The abnormal promyelocytesfrequently have bi-lobed or dumbbell shaped nuclei with granular cytoplasm. They can be difficult to recognize due to their scarcity, but when seen they should immediately raise the possibility of APL.
APML: Rx
all-trans retinoic acid (ATRA; also known as tretinoin)
Acute promyelocytic leukemia (APML, APL) is the M3 subtype of acute myelogenous leukemia (AML), a cancer of the white blood cells.[1] In APL, there is an abnormal accumulation of immature granulocytes called promyelocytes. The disease is characterized by a chromosomal translocation involving the retinoic acid receptor alpha (RARα or RARA) gene and is distinguished from other forms of AML by its responsiveness to all-trans retinoic acid (ATRA; also known as tretinoin) therapy
CLL with AIHA: Rx
Splenectomy
Considered pathognomonic for AML, are cytoplasmic inclusions of aggregated lysosomes
Auer rods
Auer rods
Anemia in AML: why?
Leukemic infiltration of marrow
Leukemic cells infiltrate the marrow and other organs (e.g., gums, skin, central nervous system [CNS]) and suppress normal hematopoiesis, resulting in cytopenias
Mechanism of MI in AML
Hyperleukocytosis plugs up small vessels
due to increased WBC count (may be seen with WBC 35,000–50,000/mL, more common with WBC >100,000/mL) results in obstruction of capillaries and small blood vessels, causing widespread ischemic changes such as stroke and/or mental status changes, congestive heart failure and/or myocardial ischemia, pulmonary congestion and/or hypoxia, and renal failure
AML associated with DIC
APML
Purpura, RF+, low complement
Cryoglobulinemic vasculitis
Cryoglobulin (CG) consists of immunoglobulins (Ig) and complement components and precipitates upon refrigeration of serum.
Cryoglobulinemia: systemic inflammatory syndrome that involves small to medium vessel vasculitis due to CG-containing complexes.
Hyperviscosity is typically associated with CG due to hematological malignancies and monoclonal Ig, while “Meltzer’s triad” of palpable purpura, arthralgia, and myalgia is generally seen with polyclonal CGs present in essential, viral, or systemic rheumatic disease-associated CG.
Meltzer’s triad
palpable purpura, arthralgia, and myalgia
“Meltzer’s triad” of palpable purpura, arthralgia, and myalgia is generally seen with polyclonal CGs present in essential, viral, or systemic rheumatic disease-associated CG.
Idiopathic vasculitis caused by circulating CGs that contain both a polyclonal immunoglobulin G (IgG) and an IgM rheumatoid factor (RF) directed against the IgG
Mixed CG
Associated with chronic HCV infection
Complement and PAN: relationship
PAN does not cause hypocomplementemia
Tumor lysis syndrome: oliguria+hyperphosphatemia, Rx
Dialysis
TLS: uric acid lowering drug
Rasburicase
recombinant urate oxidase
is well tolerated, rapidly breaks down serum uric acid, and is effective in preventing and treating hyperuricemia and TLS [9,16-23]. This rapid reduction in serum uric acid is in contrast to the effect of allopurinol, which decreases uric acid formation and therefore does not acutely reduce the serum uric acid concentration.
CVID+lymphadenopathy
NHL
Common variable immunodeficiency (CVID) is the most common form of severe antibody deficiency affecting both children and adults.
Immune defect: impaired B cell differentiation with defective production of immunoglobulin.
CVID is defined by: low total IgG) + low IgA and/or low IgM
poor or absent response to immunization, and the absence of any other defined immunodeficiency state.
CVID: complications
- Infections
- Autoimmune conditions
- Malignancy: particularly NHL
CVID: Dx
- suggestive history
- Low total serum concentration of IgG, plus low IgA or IgM,
- poor responses to both protein- and polysaccharide-based vaccines
Preventing TACO
Blood transfusion rate: 1 ml/kg/hour
Strategies to reduce the risk of TACO:
avoiding unnecessary transfusions; transfusing only the number of units needed; limiting the infusion rates to 2 to 2.5 mL/kg per hour or to 1 mL/kg per hour in high-risk individuals); and in some cases administering a diuretic or reducing the volume of the transfused product.
TACO vs TRALI
TACO: BNP goes up
TRALI: can occur before significant volume given
TRALI: hypotension
RALI is an acute transfusion reaction characterized by respiratory distress, hypoxia, and diffuse bilateral infiltrates on chest radiography. Like TACO, patients with TRALI typically become symptomatic during, or within six hours of, a transfusion.
Unlike TACO, the risk of TRALI is not related to the volume of the transfusion. TRALI may occur closer in time to the initiation of the transfusion (before significant volume is infused) and is often associated with hypotension, fever, and transient leukopenia. TRALI is not associated with an elevated N terminal Pro-BNP (NT Pro-BNP), central venous pressure, or pulmonary artery wedge pressure. In TRALI, the ratio of protein in the edema fluid to plasma is high, reflecting an exudate rather than a transudate
Hepatosmegaly + massive splenomegaly+bone disease+Pancytopenia, no lymphadenopathy, no leukoerythroblastic picture
Gaucher
Severe thrombocytopenia after PCI
Abciximab
GIIb/IIIa inhibitor induced thrombocytopenia
Hemolytic anemia, thrombosis, pancytopenia
Paroxysmal nocturnal hemoglobinuria
PNH: Rx
Eculizumab
PNH: why is it hard to diagnose?
Peculiar features
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disorder with an unusual constellation of clinical findings. The rarity of the disease and nonspecific clinical features can result in significant delays in diagnosis. The importance of a prompt and accurate diagnosis has increased as effective therapies have become available, and diagnostic testing has evolved significantly as the molecular basis of the disease and pathogenesis of hemolysis become better understood.
MGUS vs MM
- MGUS: M-protein < 3
- Asymptomatic multiple myeloma: M-protein > 3
- Multiple myeloma: M-protein > 3 + evidence of end-organ damage, eg lytic lesions, renal disease
Pancytopenia, splenomegaly, monocytopenia, atypical lymphocytes with radial projections
Hairy cell leukemia
MPN.PCV
Polycythemia vera: which mutation?
JAK2 V617F
95% sensitive
Leukemias.CML
CML vs AML
- AML: high blasts.
- AML: acute onset
- AML: no splenomegaly
Essential-thrombocythemia.Mx
Age > 60 or history of thrombosis
Hydroxyurea + low dose aspirin
MPN.JAK2
Significance of V1617F mutation
- ET: 50 to 60%
- PMF: 50 to 60%
- PCV: 90%
Pulmonary-Embolism.Mx
Duration of Rx for provoked PE
3 months
