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Heme Part 1 Flashcards

1
Q

Reticulocytes

A

Slightly immature RBCs

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2
Q

Nl reticulocyte count

A

0.5-1.5%

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3
Q

Plasma cell

A

A type of B lymphocyte that is differentiated

Makes all the antibodies

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4
Q

What are the two main categories of blood disorders of hematopoietic stem cells?

A 
Hematopoietic stem cells level
More differentiated level
-Myeloid
-Lymphoid
-Erythroid
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5
Q

Characteristics of aplastic anemia

A

Pancytopenia
Severely hypocellular bone marrow
Usually acquired- secondary

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6
Q

Causes of aplastic anemia

A

Toxic
Viral
Autoimmune mechanisms- dominant causes

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7
Q

Medication causes of aplastic anemia

A

NSAIDs
B-lactam antibiotics
Antiepileptic drugs
Psychotropic meds

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8
Q

Aplastic anemia tx

A 
Immunosuppression (70%)
-Cyclosporine
-Antithymocyte globulin
HSC transplantation
-Allogenic HSCT is a potentially curative therapy and should be considered for those younger than 50 yrs who have compatible donors
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9
Q

Pure red cell aplasia

A

Isolated, severe anemia without an adequate reticulocyte response
Bone marrow shows an absence or erythrocyte precursors

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10
Q

Causes of pure red cell aplasia

A

Similar to aplastic anemia including parvovirus B19

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11
Q

Dx of pure red cell aplasia

A

Requires bone marrow examination to exclude secondary causes, such as lymphoproliferative disorders

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12
Q

Tx of pure red cell aplasia

A

Immunosuppression
Prednisone
Cyclosporine
Cyclophsophamide

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13
Q

Types of neutropenia

A

Isolated
Immune-mediated
Nutritional deficiencies

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14
Q

Isolated neutropenia

A

Hereditary, toxic or immune causes

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15
Q

Immune-mediated neutropenia

A

Connective tissue diseases
-SLE or RA
Treat with antirheumatic drugs

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16
Q

Nutritional disease neutropenia

A

Vit B12

Folate deficiency

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17
Q

Myelodisplastic syndromes-MDS

A

Bone marrow is most commonly hypercellular
Full bone marrow yields low blood counts because the cells are ineffectively formed and have limited survival
Peripheral counts are low because they’re staying in the bone marrow and not going in the periphery

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18
Q

Causes of myelodisplastic syndromes

A

Primary process- more common

Secondary process- radiation or chemo

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19
Q

Severity of myelodisplastic syndromes

A

Ranges from asymptomaatic with mild normocytic or macrocytic anemia to transfusion-dependent anemia heralding conversion to AML

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20
Q

When to suspect myelodysplastic syndromes

A

Suspect in pts with macrocytic anemia or pancytopenia in whom vit B12 or folate deficiency have been excluded

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21
Q

Tx of myelodisplastic syndromes

A

Transfusions or erythropoiesis-stimulating agents
Prevent transformation to AML
-If pt has complex cytogenetics and a marrow blast count of greater than 10% __________
-Allogenic HSCT or hypomethylating chemo

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22
Q

Myeloproliferative neoplasms

A

Acquired genetic defects in myeloid stem cells that have deregulated production of leukocytes, erythrocytes, or platelets

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23
Q

Types of myeloproliferative neoplasms

A 
Chronic myeloid leukemia (CML)
-Too many white cells
Polycythemia vera (PV)
-Too many red cells
Essential thrombocytopenia (ET)
-Too many platelets
Primary myelofibrosis
Eosinophilia and hypereosinophilic syndrome
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24
Q

Polycythemia vera

A 
D/o of the myeloid/erthyroid stem cell
-Mutation of JAK2 (JAK2 V617F)-97% present
Hgb >18.5 g/dL in men
Hgb >16.5 g/dL in women
Must r/o secondary causes
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25
Sx of secondary polycythemia vera- hypoxemia
Thrombosis, TIA | Erythromelalgia unlikely, pruritis unlikely
26
Conditions in which secondary polycythemia vera can happen
Sleep apnea COPD Congenital heart disease Severe renal artery stenosis
27
PE of secondary polycythemia vera
Plethora | No splenomegaly
28
Lab studies of secondary polycythemia vera
No basophilia, no leukocytosis JAK 2 negative HIGH epo
29
Sx of polycythemia vera
Pruritis after a warm shower Erythromelalgia TIA DVT/PE
30
PE of primary polycythemia vera
Splenomegaly | Plethora
31
Lab studies of primary polycythemia vera
Basophilia, leukocytosis, thrombocytosis JAK 2 pos LOW epo
32
Tx of polycythemia vera
Low-dose ASA Phlebotomy -Goal of Hct <45% Hydroxyuria- antimetabolite chemotherapeutic agent
33
Prognosis of polycythemia vera
5-10% evolve into AML
34
Essential thrombocytopenia
Suspect when platelets >600,000 on 2 occasions at least 1 mo apart in the absence of secondary causes (IDA, infections, etc) JAK 2 mutation is present in 50% of pts Philadelphia chromosome must also be excluded
35
Sx of essential thrombocytopenia
``` Asymptomatic Digital ischemia Erythromelalgia TIA Visual disturbances Venous thromboembolism Bleeding ```
36
International prognostic score for essential thrombocytopenia
Low- age <60, no thrombosis, WBC <11,000 | High- score of 3-4
37
Tx of essential thrombocytopenia
Low risk: low-dose ASA High risk: platelet-lowering therapy -Hydroxyurea -Anagrelide: may exacerbate heart failure -Interferon alpha: only agent safe in pregnancy Plateletpheresis- temporary decrease -Can be done in the hospital
38
Primary myelofibrosis
Abnl, proliferating megakaryoctyes that produce excess fibroblast growth factor No dominant blood count JAK2 present in 50% Extramedullary hematopoiesis -Blood production in sites that don't usually make blood --LNs, bone marrow, spleen, liver: places that usually make blood
39
Sx of primary myelofibrosis
Asymptomatic Cytokine-mediated sx -Fever, chills, night sweats, malaise Early satiety, weight loss, abd discomfort
40
Tx of primary myelofibrosis
Hydroxyurea Ruxolitinib- first JAK2 inhibitor Allogenic hematopoietic stem cell transplantation
41
Eosinophilia and hypereosinophilic syndromes
Eosinophilia >1500/microL and tissue infiltrates
42
Causes of eosinophilia and hypereosinophilic syndromes
``` Collagen vascular dz Helminthic infections Idiopathic Neoplasia (lymphomas MC) Allergy, atopy, asthma (carbamazepine, sulfonamides) ```
43
Tx of eosinophilia and hypereosinophilic syndromes
Glucocorticoids (lytic effect) | Imatinib
44
G-CSF
Stands for granulocytic colony stimulating factor Used to stimulate production of neutrophils in autoimmune neutropenia, to hasten neutrophil recovery after cytotoxic chemo, and for HSC mobilization
45
Recombinant erythropoietin
Indicated for chemo-associated anemia and CKD anemia | Target hemoglobin level of no more than 11 g/dL
46
Allogenic HSCT
HLA-matched sibling or matched unrelated donor Chemo-less intense Immunosuppression Peripheral blood infusion of HSc Donor immune cells recognize the pt's cancer cells as foreign and mount T-cell/NK-cell mediated attack
47
What is an HSCT most helpful in treating?
Aplastic anemia High-risk MDS Acute leukemias
48
What are the risks of an HSCT?
``` Opportunistic infection -Aspergillus -Different viral and fungal infections Graft-versus-host dz (GVHD) -Severe treated with glucocorticoids ```
49
Plasma cell dyscrasias
Clonal plasma or lymphoplasmacytic cell disorders characterized by the production of monoclonal antibody (M protein) detectable in serum or urine
50
Plasma cell disorders
Monoclonal gammopathy of undetermined significance (MGUS) -Not full-blown multiple myeloma Multiple myeloma
51
B-cell disorders
Waldenstrom macroglobulinemia | Chronic lymphocytic leukemia/small lymphocytic lymphoma
52
Lab diagnostics of multiple myeloma and related disorders
``` CMP (calcium, creatinine, albumin) CBC SPEP/UPEP Serum or urine immunofixation Serum FLC (free light chain testing) ```
53
Definition of monoclonal gammopathy of undetermined significance (MGUS)
M protein level < 3 g/dL Clonal plasma cells comprising less than 10% of the bone marrow cellularity Absence of PCD-related signs and sx
54
F/u of monoclonal gammopathy of undetermined significance
``` Reevaluate yearly with: CBC Serum calcium Creatinine Repeat M protein testing 1% risk of transformation into multiple myeloma yearly (or other conditions) ```
55
Multiple myeloma
Plasma cell malignancy involving the bone marrow
56
Asymptomatic multiple myeloma
Smoldering disease | M protein >3 g/dL or >10% bone marrow plasma cells
57
Symptomatic multiple myeloma
``` End-organ damage Fatigue Anemia Rouleaux formation Leukopenia Osteopenia Kidney dysfunction ```
58
CRAB criteria for myeloma-related s/sx
HyperCalcemia: >11 mg/dL Renal failure: serum Crt >2 mg/dL Anemia: Hgb <10 g/dL Bone disease: lytic bone lesion
59
Additional diagnostic for multiple myeloma
``` Skeletal survey -X-ray -CT -MRI Bx -Bone marrow - +/- kidney biopsy ```
60
Tx for multiple myeloma
Induction chemo Autologous hematopoietic stem cell transplantation Surgical stabilization for pathologic fxs Surgical stabilization for pathologic fxs Pamidronate or zoledronic acid once very 3-4 wks for a minimum of 2 yrs in new pts with symptomatic myeloma Hypercalcemia tx Vaccinations- influenza, pneumococcal
61
Anemia
Decreased circulating RBC mass or hemoglobin Results from blood loss or underproduction or destruction of erythrocytes Anemia is not a dx but a sign of an underlying condition
62
General anemia s/sx
Fatigue Dizziness SOB Palpitations
63
Microcytic anemia
MCV <80 Iron deficiency Thalassemia
64
Normocytic anemia
Anemia of kidney dz Inflammatory anemia Hereditary spherocytosis Sickle cell dz
65
Marcrocytic anemia
``` MCV >100 Vit B12 or folate deficiency Liver dz Hypothyroidism Myelodysplastic syndrome Autoimmune hemolytic anemia ```
66
Iron deficiency anemia
Iron is absorbed in the duodenum | The most common nutritional deficiency worldwide
67
Causes of iron deficiency anemia
Loss of iron- bleeding Decrease uptake- decrease absorption Increase requirements- pregnancy
68
Ferritin in iron deficiency anemia
Low with iron deficiency anemia | May be in the nl range when associated with inflammatory conditions (RA, malignancy)
69
Reticulocytes in iron deficiency anemia
Reflects bone marrow response to anemia
70
Tx of iron deficiency anemia
Treat underlying cause Replace iron -Oral supplementation takes mos to correct, GI SEs -IV infusion: good if malabsorption issues -Erythrocyte transfusion: only if profoundly anemic and symptomatic
71
Oral replacement for iron deficiency anemia
Ferrous sulfate: 65 mg per 325 mg tablet Ferrous gluconate: 36 mg per 300 mg tablet Ferrous fumarate: 33 mg per 100 mg tablet
72
IV replacement for iron deficiency anemia
Ferric gluconate: 12.5 mg/mL Iron dextran: 50 mg/mL Iron sucrose: 20 mg/mL Ferumoxytol: 30 mg/mL
73
Inflammatory anemia
``` AKA: anemia of chronic dz Common in hospitalized pts Infection, cancer, autoimmune diseases -Chronic heart failure, DM Usually mild Often normocytic with a low TIBC ```
74
Anemia of kidney disease
Very common with CKD | -Affects 90% of pts with GFR <30
75
Components of anemia of kidney dz
Decreased epo Decreased lifespan of RBC Blood marrow suppression (from uremic toxins) and blood destruction during hemodialysis Usually normochromic and normocytic with low retic count
76
Vitamin B12 deficiency
Cofactor needed by 2 enzymes in human cells | Deficiency causes increased methylmalonic acid and homocysteine levels and affects myelopoiesis
77
What does vit B12 deficiency result in?
Macrocytic anemia | Demyelinating nervous system disease
78
Risk factors for vit B12 deficiency
``` Vegetarians Absorption issues -IBD -Gastric -Bariatric or ileal surgery Pernicious anemia -MCC of severe deficiency -Destruction of gastric parietal cells- makes intrinsic factor Increased with autoimmune conditions -Thyroid dz -DM -Vitiligo ```
79
S/sx of vitamin B12 deficiency
``` Anemia Neurologic dysfunction Glossitis Hyperpigmentation Infertility ```
80
Neurologic dysfunction in vit B12 deficiency
``` Symmetric paresthesias Numbness Decreased vibratory sense Gait problems Neuropsychiatric sx ```
81
Peripheral blood smear for vit B12 deficiency
Large oval erythrocytes Hypersegmented neutrophils Possible pancytopenia
82
Other tests for vit B12 deficiency
Cobalamin level Methylmalonic acid Homocysteine level
83
Tx of vit B12 deficiency
Oral replacement -1000 to 2000 micrograms/d Cobalamin injections -IM or SC -1,000 micrograms several times per week for 1-2 wks, then weekly until symptom relief or improved findings -Monthly injections thereafter if pernicious anemia -Usually corrects in 6-8 wks
84
Folate deficiency
Megaloblastic anemia with impaired DNA synthesis
85
Causes of folate deficiency
``` Poor intake Alcoholics Malabsorption Meds that accelerate folate metabolism -Phenytoin -Trimethoprim -Methotrexate ```
86
Lab findings of folate deficiency
Similar to cobalamin deficiency, except methylmalonic acid levels will NOT be elevated
87
Tx of folate deficiency
Oral folate 1-5 mg/d
88
Categories of hemolytic anemias
Inherited or acquired | Intravascular or extravascular
89
Findings in hemolytic anemias
Compensatory reticulocytosis (usually) Elevated indirect bilirubin and LDH Free hemoglobin secretion (hemoglobinuria)
90
Sx of hemolytic anemias
``` Anemia Jaundice Dark urine Cholelithiasis- chronic hemolysis Extramedullary hematopoiesis ___________ LAD ```
91
Hereditary spherocytosis
Mostly autosomal dominant (75%) Osmotically fragile spherocytes Splenomegaly
92
Lab testing for hereditary spherocytosis
Osmotic fragility testing | Cryohemolysis, eosin-5-malemide binding
93
Tx for hereditary spherocytosis
Folic acid supplementation | Splenectomy with vaccinations prior strep pneumo, hib, and meningococcal
94
G6PD deficiency
X-linked disorder | Most common enzyme deficiency in humans
95
Hemolysis triggers for G6PD deficiency
Sulfonamides, nitrofurantoin Antimalarials Infection
96
Lab findings in G6PD deficiency
``` Bite cells Heinz bodies -Denatured oxidized hemoglobin Fluorescent spot screening test Quantitative testing ```
97
Tx of G6PD deficiency
Avoid offending agents | Supportive +/- blood transfusions
98
Thalassemia
Mutation in either the alpha or beta globin At least 1-5% of the world has a mutation Most commonly in Mediterranean, Middle East, southeast Asia
99
Lab findings in thassemia
Microcytic Nl iron studies Target cells Electrophoresis
100
Tx of thalassemia
Trait- education Oral folic acid 1 mg/d Hemoglobin H dz (deletion of 3 alpha genes) -Occasional blood transfusions --Higher risk of iron overload Allogenic hematopoietic stem cell transplantation can be curative and should be considered with severe forms before the onset of end-organ damage
101
Sickle cell syndromes
Hemoglobinopathy with mutation in the B-globin chain
102
Pathophysiology of sickle cell syndromes
``` Oxidative stress Adhesion of cells to the endothelium Inflammation Decreased nitric oxide Vasoconstriction ```
103
Complications of thalassemia
Chronic hemolytic anemia Infections- parvovirus B19, vaccines Vit deficiencies- folate ACS, PE, ischemic stroke, pulm HTN, CKD, hepatic crisis
104
Tx of thalassemia
HSCT Prophylactic transfusions Hydroxyurea therapy- reduces vaso-occlusive episodes Chronic pain
105
Primary hemochromatosis
Autosomal recessive defect | Affects 1 in 400 persons of N. European ancestry
106
S/sx of primary hemochromatosis
``` Chronic fatigue Weakness Nonspecific abd pain Athralgia Mildly elevated liver enzymes +/- hypothyroidism DM Gonadal failure Eventual hepatic fibrosis and chirrhosis (HCC) ```
107
Lab findings in primary hemochromatosis
Fasting serum transferrin saturation - >60% in men, >50% in women - Elevated ferritin
108
Tx of primary hemochromatosis
Phlebotomy- 1 unit weekly until ferritin levels decrease to 10-50 ng/mL Iron chelation if phlebotomy is contraindicated
109
Platelet physiology
Made in the bone marrow from megakaryocytes Lifespan is around 10 days Nl range: 150K-450K/microL Hormonal stimulus is thrombopoietin Form the initial plug at the site of vascular injury -Adhesion, aggregation, secretion Activated glycoprotein IIb-IIIa allows the fibrinogen binding
110
Thrombocytopenia sx
Mucocutaneous bleeding- epistaxis, gum bleeding, heavy menses, easy bruising, petechiae Seldom seen if platelet count >30K
111
Hx in thrombocytopenia
``` Medications TTP, HUS ITP, HIT Use hematology: purple top Sodium citrate anticoagulant ```
112
Meds associated with drug-induced thrombocytopenia
``` Anticonvulsants Anti-inflammatory agents Antimicrobials CV drugs Chemotherapeutic agents Glycoprotein IIb/IIIa inhibitors Haloperidol Ranitidine Simvastatin ```
113
What are the three causes of thrombocytopenia?
Disorders of underproduction Peripheral destruction Splenic sequestration
114
Disorders of underproduction in thrombocytopenia
Marrow failure- aplastic anemia, B12/folate deficiency Marrow invasion- leukemias Marrow injury- drugs (EtOH, chemo), radiation
115
Peripheral destruction in thrombocytopenia
Immune or non-immune mechanisms
116
Non-immuned mediated thrombocytopenia
Thrombotic Thrombocytopenia Purpura (TTP) | Hemolytic Uremic Syndrome (HUS)
117
Immune-mediated (platelet antibodies) thrombocytopenia
Immune Thrombocytopenia Purpura (ITP) | Heparin-Induced Thrombocytopenia (HIT)
118
TTP
Congenical or acquired deficiency of protease ADAMTS-13 Abnl activation of platelets and endothelial cells, deposition of fibrin in microvasculature, and peripheral destruction of RBC and platelets
119
Clinical pentad of TTP
``` Thrombocytopenia Anemia- Microangiopathic Hemolytic Anemia (MAHA) Fever Kidney disease Neurologic findings Abd pain ```
120
Additional laboratory findings of TTP
Elevated bilirubin and LDH | Schistocytes
121
Tx of TTP
Emergent plasma exchange | Platelet transfusions are contraindicated
122
HUS
Frequently overlaps with TTP Occurs more frequently in children -Has MORE kidney and fewer neurologic manifestations Can be precipitated by infectious diarrheal illnesses -E. coli 0157:H7
123
Tx for HUS
Plasma exchange
124
ITP
Suspect with isolated new-onset thrombocytopenia
125
Causes of ITP
Autoimmune conditions Leukemia/lymphoma Viral illnesses (Hep C, HIV) Pregnancy
126
What to r/o in ITP
Splenomegaly and thyroid dz | ANY new meds, supplements
127
Tx of ITP
Reserve for platelet counts <30K or if the pt is bleeding Glucocorticoids -Prednisone 1 mg/kg then taper over weeks -Dexamethasone 40 mg/d x 4 days IVIG Splenectomy Off-label rituximab
128
HIT
Drug-induced with antibody against the heparin/PF4 complex that can activate platelets causing thrombocytopenia +/- thrombosis Platelet counts decrease by at least 50% 5-10 days after tx with heparin
129
Lab testing for HIT
Serology for anti-heparin/PF4 antibodies
130
Tx of HIT
STOP heparin Begin Argatroban therapy Baseline doppler u/s of all 4 extremities Do not start warfarin therapy until platelet 100-150K Pt education regarding heparin
131
Acquired platelet dysfunction
Suspect in pts with clinical bleeding whose platelet count, PT, and aPTT are nl Abnl bleeding time or platelet function analyzer- 100 result Some disorders respond to desmopressin, whereas others require transfusions
132
Causes of acquired platelet dysfunction
``` Uremia Liver dz Myeloproliferative neoplasms Post-cardiac bypass Antiplatelet drugs -IIb-IIIa inhibitors -ASA -Clopidogrel -NSAIDs: temporary ```

CAM III (12 decks)

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