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MDC01 > H > Flashcards

H Flashcards

1
Q

What is polycythemia?

A

A disease state in which the proportion of blood volume occupied by RBCs increases (e.g. increased Hct or Hb levels).

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2
Q

What is relative polycythemia?

A

Decreased plasma volume with normal RBC mass – gives appearance of increased Hb or Hct. Will be associated to normal EPO level without hypoxia.

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3
Q

How is the amount of red blood regulated?

A

Erythropoietin

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4
Q

What can cause relative polycythemia?

A

Diuretics, severe dehydration, burns, smoking, obesity, Gaisbock syndrome (state of chronically reduced plasma vol w/ inc. Hb or Hct).

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5
Q

What is absolute polycythemia?

A

Erythrocytosis – increased RBC mass in presence of normal plasma volume.

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6
Q

How is absolute polycythemia further categorized?

A

Primary (low or normal EPO) and secondary (high EPO).

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7
Q

What is primary polycythemia?

A

An acquired or inherited mutation leading to abnormality within RBC progenitors (e.g. polycythemia vera).

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8
Q

What is polycythemia vera?

A

A myeloproliferative blood disorder in which marrow makes too many RBCs +/- inc. production of WBCs and platelets.

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9
Q

What is secondary polycythemia?

A

Increased production of RBCs caused by natural or artificial inc. in EPO.

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10
Q

List some conditions which may result in a physiologically appropriate secondary polycythemia.

A
  • poor tissue oxygenation/hypoxia
  • pulmonary disease: COPD, sleep apnea, pulmonary hypertension
  • cardiovascular disease
  • RBC/Hb defects (Hb w/ inc. O2 affinity)
  • CO poisoning (e.g. heavy smoking)
  • high altitude
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11
Q

List some conditions where secondary polycythemia is not a result of physiologic adaptation.

A
  • renal cell carcinoma, cerebellar hemangioblastoma, hepatocellular carcinoma, uterine leiomyomas, ovarian tumor
  • other causes: polycystic kidney disease, post-kidney transplant, hydronephrosis, androgens, exogenous erythropoietin (performance-enhancing drugs)
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12
Q

What are the clinical features of polycythemia?

A
  • inc. Hct and Hb
  • hyperviscosity
  • headache, dizziness, tinnitus (perception of sound in absence of actual sound), visual disturbances (e.g. double vision)
  • thrombosis (venous or arterial) or bleeding (abnormal platelet function)
  • splenomegaly +/- hepatomegaly, facial plethora (ruddy complexion) (70%) and/or palms, gout
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13
Q

How is polycythemia investigated?

A

Serum erythropoietin (EPO):

  • inc. suggest autonomous production or hypoxia (rules out polycythemia vera)
  • search for tumor as source of EPO (e.g. abdominal U/S, or CT head)
  • JAK-2 mutation analysis: positive in > 96% of cases PV
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14
Q

How do you treat secondary polycythemia?

A

O2 for hypoxemia, surgery for EPO-secreting tumors

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15
Q

What are the clinical features of polycythemia vera (PV)?

A
  • inc. Hct and Hb
  • hyperviscosity
  • thrombotic complications (e.g. DVT, PE) due to inc. blood viscosity and inc. platelet count/activity
  • erythromelalgia (burning pain in hands and feet and erythema of skin)
  • pruritus (due to mast cell degranulation and histamine release)
  • epigastric distress, PUD
  • gout
  • splenomegaly +/- hepatomegaly
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16
Q

How is PV investigated?

A
  • must rule out secondary polycythemia
  • diagnosis requires 2 major + 1 minor OR 1st major + 2 minor:

1M. Hb > 185 men or 165 fem

2M. presence of JAK2V617F or other mutation

1m. bone marrow biopsy showing hypercellularity w/ panmyelosis
2m. serum EPO below normal
3m. endogenous erythroid colony formation in vitro

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17
Q

How is PV treated?

A
  • phlebotomy to keep Hct < 45%
  • low-dose aspirin (antithrombotic prophylaxis, also treat erythromelalgia)

CANNOT BE CURED

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18
Q

What is neutropenia?

A

Put simply, it is a decrease in the number of circulating neutrophils.

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19
Q

What are three MAIN causes of neutropenia?

A
  1. decreased production
  2. increased destruction
  3. excessive margination (movement out of circulatory system)
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20
Q

What is an example of a congenital syndrome that causes severe neutropenia?

A

Kostmann syndrome.

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21
Q

What is Kostmann syndrome?

A

An autosomal recessive form of severe chronic neutropenia.

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22
Q

How is Kostmann syndrome treated?

A

G-CSF (granulocyte colony-stimulating factor)

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23
Q

What diseases are patients at increased risk of developing while using G-CSF?

A

Myelofibrosis and acute myeloid leukemia (AML).

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24
Q

What is the most frequent cause of neutropenia in adults?

A

Drug-induced

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25
Q

What are the 5 anti- drugs associated to neutropenia?

A

anti-biotics, anti-inflammatories, anti-psychotics, anti-seizure, anti-thyroid

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26
Q

What are the 2 important classifications of lymphadenopathy?

A

Localized vs diffuse

Reactive (inflammatory) vs neoplastic

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27
Q

Differentiate between a reactive (inflammatory) node and a neoplastic one in terms of lymphadenopathy (categories are consistency, mobility, tenderness, size).

A

Consistency: rubbery (R/I), firm/hard (N)

Mobility: mobile (R/I), matted/immobile (N)

Tenderness: tender (R/I), non-tender (N)

Size: <2 cm (R/I), >2 cm (N)

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28
Q

What is sarcoidosis?

A

A disease in which inflammation occurs in the lymph nodes, lungs, and other tissues.

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29
Q

What differentiates Hodgkin’s disease from non-Hodgkin lymphomas?

A

The presence of Reid-Sternberg cells.

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30
Q

From what cell line do Reed-Sternberg cells usually arise?

A

B-cells

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31
Q

What is the most common clinical presentation of lymphoid malignancy?

A

Painless enlargement of lymph nodes (lymphadenopathy).

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32
Q

If the cause of persistent lymphadenopathy is not apparent after thorough evaluation, what should be done?

A

Biopsy.

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33
Q

An enlarged lymph node in this area is highly suggestive of malignancy and should always be sampled.

A

Supraclavicular.

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34
Q

What should be done if a lymph node biopsy is nondiagnostic and unexplained lymph node enlargement persists?

A

Biopsy should be repeated.

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35
Q

TRUE/FALSE: The cause of most NHLs is not known.

A

True.

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36
Q

What are B symptoms?

A

Fever, night sweats, weight loss

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37
Q

How many stages are there in the Ann Arbor Staging System?

A

4 (four).

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38
Q

Describe each stage of the Ann Arbor Staging System.

A

Stage I - involvement of single lymph node region/extralympatic organ/site

Stage II - involvement of 2 or more lymph region OR extralymphatic site and 1 or more lymph node regions on same side of diphragm

Stage III - involvement of lymph node regions on both sides of diaphragm

Stage IV - diffuse involvement of on or more extralymphatic organs

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39
Q

t(8;14)

A

Burkitt’s lymphoma

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40
Q

t(14;18)

A

Follicular lymphoma

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41
Q

t(9;22)

A

CML, sometimes ALL (Philadelphia chromosome)

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42
Q

t(11;14)

A

mantle cell lymphoma

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43
Q

In what type of leukemia are Auer rods present in?

A

Acute myeloid (NOT lymphoid). Also, Auer may or may not be present in myeloid but are NEVER in lymphoid.

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44
Q

What is AML (acute myeloid leukemia)?

A

Rapidly progressive malignancy characterized by failure of myeloid cells to differentiate beyond BLAST stage.

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45
Q

Why is anemia a clinical feature of AML?

A

Suppression of normal hematopoietic cells due to uncontrolled growth of blasts in marrow.

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46
Q

Label all the blanks with their corresponding blood cell malignancy.

A

AML: myeloid stem cell

CML: pluripotent stem cell

ALL: lymphoid stem cell

CLL: B lymphocyte

MM: plasma cell

Lymphoma: bracket on right

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47
Q

In which type of leukemia are the genes regulating maturation INTACT?

A

CLL

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48
Q

What is Richter’s transformation?

A

A rare complication of CLL (or follicular cell lymphoma) in which the leukemia changes into a fast-growing diffuse large B-cell lymphoma.

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49
Q

What are the five categories of immune deficiency disorders?

A
  1. antibody (humoral)
  2. combined antibody and cellular
  3. phagocytes
  4. isolated cellular
  5. complement system
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50
Q

What are B-symptoms?

A

weight loss, fever, night sweats

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51
Q

What is the most common adult leukemia?

A

CLL (chronic lymphocytic leukemia)

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52
Q

What is CLL (chronic lymphocytic leukemia)?

A

Indolent disease characterized by clonal malignancy of mature B-cells.

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53
Q

What are the different Rai stages of CLL?

A

0: bone marrow and blood lymphocytosis only
1: lymphocytosis with enlarged nodes
2: lymphocytosis with enlarged spleen
3: lymphocytosis anemia
4: lymphocytosis with thrombocytopenia

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54
Q

What is Richter’s transformation?

A

When CLL evolves into diffuse large B-cell lymphoma.

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55
Q

______cytic:

  1. MCV < 80
  2. MCV = 80-100
  3. MCV > 100
A
  1. Micro
  2. Normo
  3. Macro
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56
Q

Hypochromic RBCs are associated to what 3 anemias?

A

iron deficiency anemia, anemia of chronic disease, sideroblastic anemia

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57
Q

What are Reed-Sternberg cells?

A

Giant, multinucleated B-lymphocyte associated with Hodgkin lymphoma

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58
Q

A nucleus is present in what kind of RBCs and what conditions?

A

Erythroblasts (immature RBCs) from hyperplastic erythropoiesis, BM infilitration, MPNs

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59
Q

What is the RBC inclusion in Heinz bodies?

A

Denatured and precipitated Hb

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60
Q

What conditions are associated to Heinz bodies?

A

G6PD (post-exposure to oxidant), thalassemia, unstable HB

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61
Q

What are Howell-Joly bodies?

A

RBCs with small nuclear remnants of nucleus.

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62
Q

What conditions are Howell-Joly bodies associated with?

A

Splenectomy, SCD, megaloblastic anemia

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63
Q

What are basic investigations to carry out in any kind of anemia?

A

CBC with differential, retic count, blood film

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64
Q

What are causes of microyctic anemia?

A

Iron deficiency, thalassemia, Pb poisoning, anemia of chronic disease, sideroblastic anemia

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65
Q

Thrombocytopenia is caused by three different things…what are they?

A
  1. Decreased production
  2. Increased destruction
  3. Sequestration
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66
Q

What are clinical features of thrombocytopenia?

A

Bruising, petechiae, ecchymoses

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67
Q

What are typical investigations for thrombocytopenia?

A

CBC with diff, blood film, work-up for B12 and folate

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68
Q

How do you treat life threatening bleed with thrombocytopenia?

A

Platelet transfusion.

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69
Q

What is thrombocytosis?

A

TOO MANY PLATELETS!

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70
Q

What is primary thrombocytosis caused by?

A

Myeloproliferative neoplasm (e.g. CML, primary myelofibrosis, polycythemia vera)

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71
Q

What is reactive/secondary thrombocytosis caused by?

A

Acute phase reactant (e.g. surgery, inflammation, infection, trauma, IRON DEFICIENCY, etc.)

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72
Q

How is thrombocytosis investigated?

A

CBC, peripheral blood, rule in/out reactive process if out bone marrow biopsy for MPN/MDS

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73
Q

How is primary thromobocytosis treated?

A

ASA

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74
Q

How is secondary thromobocytosis treated?

A

Treatment of underlying cause.

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75
Q

What is pancytopenia?

A

A decrease in all hematopoietic cell lines.

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76
Q

How is pancytopenia investiated?

A

CBC and diff, blood film, often requires bone marrow biopsy to determine cause

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77
Q

What is neutrophilia?

A

An overabundance of neutrophils.

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78
Q

Mature neuts or bands > 20% of total WBC suggests what?

A

Infection/inflammation

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79
Q

Neutropenia is what?

A

A lack of neutrophils.

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80
Q

What are the three grades of neutropenia?

A

Mild, moderate, severe.

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81
Q

What are the two categories of neutrophilia?

A

Primary and secondary (caused by something else, e.g. smoking)

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82
Q

Hereditary neutrophilia is autosomal dominant or recessive trait?

A

Dominant

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83
Q

Is CML an example of primary or secondary neutrophilia?

A

Primary

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84
Q

Is reactive neutrophilia secondary or primary?

A

Secondary.

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85
Q

Which classification of neutrophilia has an orderly differential: primary (neoplastic) or secondary (reactive)?

A

Secondary (reactive).

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86
Q

What is meant by an orderly differential (WBCs)?

A

Polymorphs (mature neuts) > Bands > Metamyelocytes

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87
Q

Should you avoid a DRE on patients with neutropenia?

A

Yes.

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88
Q

How do you differentiate DIC from primary fibrinolysis?

A

by platelet count! DIC involves both clotting factor and fibrinolytic system. Primary fibrinolysis involves only fibrinolytic system therefore coag abnormality is similar to DIC but platelet count remains normal.

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89
Q

Lupus inhibitor are associated with an increased risk of…

A

Thromboembolism. Lupus inhibitors are antibodies that do not affect assembly of clotting factors so coag proceeds normally, so no bleeding problem.

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90
Q

How is vit K absorption affected by antibiotics?

A

Antib wipe out normal flora in GI which are needed to absorb vit K.

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91
Q

What organs are affected by GVHD?

A

skin, liver, and gut resulting in rash, elev liver enzymes/jaundice, and diarrhea

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92
Q

How do you differentiate between leukemoid reaction and CML?

A

LmR: orderly, thrombocytosis

CML: disorderly, baso/eosin, splenomegaly, thrombocytosis, Phili Chr

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93
Q

Multiple myeloma is also known as ______ .

A

plasma cell myeloma.

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94
Q

What is multiple myeloma?

A

A neoplastic proliferation of a clone of plasma cells producing a monoclonal immunoglobulin.

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95
Q

What would a serum protein electrophoresis (SPEP) show for MM?

A

A large spike in the gamma area from extensive clonal Igs.

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96
Q

What can cause a polyclonal gammopathy?

A

90% of the time, infection.

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97
Q

What is the most common cause of monoclonal gammopathy?

A

MGUS (monoclonal gammopathy of undetermined significance)

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98
Q

What are other causes of monoclonal gammopathy (besides MGUS)?

A

multiple myelona, Waldenstrom’s macroglobulinemia, NHL, CLL, amyloidosis

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99
Q

Does multiple myeloma affect older or younger populations?

A

Older (median age of diagnosis 68). M>F

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100
Q

What does CRAB stand for in MM?

A

hyperCalcemia

Renal failure

Anemia

Bony lesions

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101
Q

What is anemia with rouleaux?

A

Stacking of RBCs due to excessive protein in the blood.

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102
Q

Is anemia in MM micro, normo, or macro?

A

Usually normocytic anemia.

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103
Q

What do malignant plasma cells in MM secrete?

A

Monoclonal antibodies (M proteins)

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104
Q

What are M proteins?

A

monoclonal Ig (identical heavy chain + identical light chain)

OR

identical light chains only

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105
Q

What are the clinical features of MM?

A

Bone disease - pain, tenderness, and pathologic fractures (from little stress)

CRAB (hyperCalcemia, Renal failure/disease, Anemia, Bony lesions)

Weight loss

Infections (suppression of normal plasma cell function)

Bleeding (thrombocytopenia)

106
Q

What are the tests/investigations for MM?

A

CBC - normocytic anemia (rouleaux formation), thrombocytopenia, leukopenia

biochemistry - inc Ca2+, inc ESR, 24-hr urin for protein

monoclonal proteins - SPEP, UPEP, immunofixation (identifies Ig type)

bone marrow aspirate/biopsy - > 10% even 30% plasma cells, cytogenetics

skeletal x-rays - lytic lesions

107
Q

What are the 3 criteria for diagnosis of MM?

A
  1. serum/urinary monoclonal protein
  2. presence of clonal plasma cells in marrow
  3. presence of end-organ damage (e.g. renal failure, lytic bone lesions, inc serum Ca2+, anemia)
108
Q

Is MM curable?

A

No.

109
Q

What is the treatment for MM?

A

Stem cell transplant < 65 y w/ corticosteroids and proteosome inhibitors

Chemotherapy > 65 w/ corticosteroids

110
Q

What is the difference in plasma cell concentration in marrow between MGUS and MM?

A

MGUS < 10%

MM > 10%

111
Q

What is MGUS (monoclonal gammopathy of unknown significance) and how does it present?

A

Presence of M protein in serum in absence of clinical or lab evidence of plasma cell or lymphoproliferative disorder. DIAGNOSIS OF EXCLUSION

ABSENCE OF CRAB

Asymptomatic

112
Q

What are amyloids?

A

Insoluble fibrous protein aggregates.

113
Q

What is amyloidosis?

A

The abnormal deposit of amyloid proteins (insoluble fibrous proteins) in organs and tissues and cause harm.

114
Q

In MM, what causes amyloidosis?

A

Deposit of lambda light chain.

115
Q

What is Waldenstrom’s macroglobulinemia?

A

An indolent type of non-Hodgkin lymphoma affecting B cells producing monoclonal IgM.

116
Q

Does MM have lymphadenopathy?

A

No.

117
Q

What are significant clinical features of Waldstrom’s macroglobulinemia?

A

Fatigue, anemia, weight loss, and HYPERVISCOSITY. Lymphadenopathy and splenomegaly are common.

Monoclonal protein is IgM.

(Hyperviscosity due to size of IgM drawing fluid)

118
Q

What are two broad categorical causes of hypogammaglobulinemia?

A
  1. Decreased production
  2. Increased loss
119
Q

What are some possible causes of increased loss of immunoglobulins?

A
  1. Burns
  2. Protein losing enteropathy
  3. Nephrotic syndrome
120
Q

Which Ig is first responder?

A

IgM

121
Q

Which Ig is the memory antibody?

A

IgG

122
Q

Which Ig is in mucous membranes?

A

IgA

123
Q

Which Ig fights parastic infections and plays a role in allergic response?

A

IgE

124
Q

Where in the body could Ig be lost or secreted?

A

GI and kidneys (urine)

125
Q

X-linked gammaglobulinemia is what type of immunodeficiency?

A

Humoral

126
Q

What immunodeficiency has no Ig present?

A

X-linked immunoglobulinemia

127
Q

What is dysgammaglobulinemia?

A

Normal Ig present, but lack specific antibody response.

128
Q

If RBCs and platelets are low what type of anemia should be considered?

A

MAHA (microangiopathic hemolytic anemia)

129
Q

What are spur cells associated to?

A

Severe liver disease (spur cell anemia), starvation/anorexia, or post-splenectomy.

130
Q

What is a Rouleaux formation?

A

RBC stacking due to inc. plasma concentration of proteinc (e.g. multiple myeloma).

131
Q

What are smudge cells?

A

Lymphocytes damaged during blood smear preparation indicating cell fragility (e.g. CLL and other lymphoproliferative disorders).

132
Q

Normocytic anemia is further divided into two broad categories and two sub-categories in each category, what are they?

A

High Retic and Low Retic

HR: Hemolysis and Bleeding

LR: Pancytopenia and Non-pancytopenia

133
Q

List the normocytic hemolytic anemias.

A

Inherited: hemoglobinopathy (e.g. SCD, thal, unstable Hb), membrane (spherocytic)

Acquired: immune (Coombs +ve, drug-related, colg agglutinin), infection (malaria), MAHA (DIC, TTP/HUS, etc.), oxidative/drug-related (G6PD)

134
Q

List your causes of normocytic pancytopenic anemias.

A

Aplastic anemia, MDS, leukemia

135
Q

What are the non-pancytopenic normocytic anemia causing diseases?

A

Anemia of chronic disease, renal/liver disease

136
Q

How is macrocytic anemia divided?

A

Megaloblastic and Non-megaloblastic anemia

137
Q

What are the two major causes of megaloblastic anemia?

A

B12 deficiency and folate deficiency

(BONUS: also drugs that impair DNA synthesis e.g. methotrexate, sulfa, chemo)

138
Q

What are causes of non-megaloblastic macrocytic anemia?

A

Liver disease, alcoholism, hypothyroidism, MDS

139
Q

What type of anemias could present with jaundice?

A

Hemolytic anemias.

140
Q

What are symptoms of anemia?

A

Faitgue, malaise, weakness, dyspnea (SOB), decreased exercise tolerance, palpitations, headache

141
Q

What investigations are carried out for anemia?

A

CBC w/ diff, retic ount, peripheral, rule out GI disease in Fe deficiency

142
Q

What is pancytopenia?

A

A decrease in all hematopoietic cell lines.

143
Q

What are the clinical features of pancytopenia?

A

Anemia - fatigue

Leukopenia - recurrent infections

Thrombocytopenia - mucosal bleeding and ecchmoses

144
Q

What test is required to determine a cause with aplastic anemia?

A

Bone marrow biopsy/aspirate.

145
Q

Why do glucocorticoids result in neutrophilia?

A

Demargination of neutrophils (neutrophils are pulled into circulation from surrounding tissue) – increases ANL but doesn’t actually benefit immune response but instead suppresses it.

146
Q

What are the three main categorical causes of neutropenia?

A
  1. Decreased production
  2. Peripheral destruction
  3. Excessive margination
147
Q

What decreased production neutropenia disorders are there?

A

Hematological disease (idiopathic, aplastic anemia, BM infiltration)

Infection (TB, EBV, viral hep., HIV)

Drug-induced (“anti-medications”)

Toxic (high dose radiation, chemicals)

Nutritional deficiency (B12, folate)

Idiopathic

148
Q

What excessive margination related causes result in neutropenia?

A

Idiopathic (most common), overwhelming bacterial infection

149
Q

What are clinical features of neutropenia?

A

Fever, chills (only in infection present)

Infection by endogenous bacteria (e.g. S. aureus, E. coli, and other G- from GI)

150
Q

What investigations can be done for neutropenia?

A

CBC with diff and possibly bone marrow asp/biopsy

151
Q

If neutropenia does not go into remission with G-CSF what treatment needs to be considered?

A

Immunosuppresion.

152
Q

What are causes of lymphocytosis?

A

Infection (viral infection majority especially mono), neoplasm (e.g. ALL, CLL, lymphoma)

153
Q

What are causes of lymphopenia?

A

idiopathic, chemo drugs, radiation, HIV, hep B and C, autoimmune disease (e.g. SLE), malignancy, malnutrition

154
Q

What are the first two etiologies that need to be considered when investigating splenomegaly?

A

CONGESTIVE: Evidence of portal hypertension or coagulopathy? (suggests cirrhosis, thrombus)

OR

HEMOLYTIC DISEASE: Blood smear abnormalities (suggests SCD, thal)

155
Q

If CONGESTIVE and HEMOLYTIC DISEASE have been ruled out in splenomegaly, what the three remaining broad categorical causes?

A

Infectious (bacterial, viral-EBV), Infiltrative (lymphoma, leukemia, PV, ET), Inflammatory (SLE)

156
Q

Where is iron absorbed in the GI?

A

Duodenum

157
Q

Why is ferritin elevated in the setting of inflammatory conditions and liver disease?

A

It is an acute phase reactant.

158
Q

What are sideroblasts?

A

RBCs with iron-containing (basophilic) granules in cytoplasm – presence is normal

Abnormal is when granules form ring around nucleus – this is sideroblastic anemia.

159
Q

Pica is a clinical feature of iron deficiency anemia, what is it?

A

An appetite for non-food substances (e.g. ice, paint, dirt).

160
Q

What is aplastic anemia caused by?

A

Destruction of hematopoietic cells of the bone marrow leading to pancytopenia and hypocellular bone marrow.

161
Q

Is aplastic anemia age specific and which sex is it most common in?

A

Not age specific.

More common in males.

162
Q

What is Fanconi’s anemia?

A

An autosomal recessive aplastic anemia mostly affecting Ashkenzai Jews.

163
Q

What are the clinical features of aplastic anemia?

A

Anemia, leukopenia, thrombocytopenia

ABSENCE of splenomegaly and lymphadenopathy

164
Q

What investigations are done for aplastic anemia?

A

CBC w/ dif, retic count (should be decreased as well), bone marrow asp/bio should show hypoplasia of marrow cells with fat replacement

165
Q

What is ABCD pneumonic for normocytic anemia?

A

Active blood loss

Bone marrow failure

Chronic disease

Destruction (hemolysis)

166
Q

What are the treatments for aplastic anemia?

A

Remove offending agents

Supportive care (RBC and platelet transfusion, antibiotics)

Immunosuppression

Bone marrow transplant

167
Q

How is hemolytic anemia classified and what are their underlying disorders?

A

Hereditary: abnormal membrane (sphero, ellipto), abnormal enzymes (G6PD), abnormal hemo (thal, others)

Acquired-Immune: Transfusion reaction, AIHA, cold agglutinins

Acquired-Non-Immune: MAHA, mechanical heart valves

168
Q

What are clinical features specific to HA (hemolytic anemia)?

A

Jaundice, dark urine, cholelithiasis (pigment stones), Fe overload extravascular hemolysis, Fe deficiency intravascular hemolysis

169
Q

How is HA (hemolytic anemia) investigated?

A

Screening tests: inc. retic, dec. haptoglobin, inc. unconj bili, inc LDH

Tests for intravascular hemolysis: shistocytes on blood film, free Hb in serum

Test for extravascular hemolysis: direct Coombs’ (anti-IgG or anti-complement, +ve if agglutinate), indirect Coombs’

170
Q

How is beta-thal minor investigated?

A

Hb electrophoresis – HbA2 inc to 2.5-5% (from 1.5-3.5%)

Will show microcytic anemia, normal Fe, normal RBC count

171
Q

What is B-thal M (beta thal major)?

A

Hemoglobinopathy resulting from defect in BOTH beta genes.

172
Q

What are the clinical features of B-thal major?

A
  1. initial presentation age 6-12 months when HbA normally replaces HbF
  2. severe anemia, jaundice
  3. stunted growth and development
  4. radiologic changes “hair on end”, pathologic fractures
173
Q

How is beta-thal major investigated?

A

CBC w/ diff, peripheral, Hb electrophoresis:

HbA 0-10% [N > 95%]

HbA2 >2.5%

HbF 90-100% (fetal)

174
Q

How is beta-thal major treated?

A
  1. lifelong regular transfusions and Fe chelation
  2. folic acid supplementation
  3. splenectomy
  4. allogenic bone marrow transplantation
175
Q

When would a transfusion be required in beta-thal minor?

A

occasionally required in pregnant women who may develop a more severe “physiologic” anemia of pregnancy

176
Q

What is alpha-thal?

A

A hemoglobinopathy affect alpha genes.

177
Q

What are the four kinds of alpha thal?

A

1 defective (of 4) gene: clinically silent, normal Hb, normal MCV

2 defective gene: decreased MCV, normal Hb

3 defective gene: HbH (B4) disease, presents in adults, dec MCV & Hb, splenomegaly

4 def: HbBarts (Gamma4) hydrops fetalis DEAD

178
Q

How is alpha thal investigated?

A

CBC w/ diff, peripheral (screen for HbH inclusion)

Hb electrophoresis NOT diagnostic for alpha-thal

DNA analysis using alpha gene probe

179
Q

What is the treatment for alpha thal?

A

1 or 2 defect: nothing

3 (HbH disease): like beta thal major (lifelong transfusion, folic acid supp, splenectomy, allogenic bone marrow xplant)

180
Q

What is SCD (sickle cell disease)?

A

A hemoglobinopathy due to mutant beta-globin chain.

181
Q

What are major pathological features of SCD?

A
  1. at low oxygen levels, HbS polymerizes leading to rigid crystal-like rods that distort membranes “sickles”
  2. sickle cells are fragile and hemolyze and obstruct small vessels
182
Q

What are the variations of severity of SCD and their clinical features?

A
  1. HbAS (sickle cell trait): mostly asymptomatic except during EXTREME hypoxia
  2. HbSS: chronic hemolytic anemia, jaundice in first year of life, stunted growth/develop, splenomegaly (child) and atrophy (adult), also HbSS presents with crisis.
  3. HbSC (most common compound heterzygote): similar to HbSS but more mild
183
Q

How is SCD investigated?

A

CBC w/ diff, peripheral, and Hb electrophoresis (distinguishes HbAS, HbSS, HbSC)

184
Q

On electrophoresis how are HbAS and HbSS differentiated?

A

HbAS has HbA 65% and HbS 35%

HbSS has HbA 0%, only HbS and HbF (proportions change with age)

185
Q

How is SCD treated?

A

HbAS no treatment required

HbSC - similar to HbSS but directed based on severity

HbSS: folic acid to prevent folate deficiency, treatment of vaso-occlusive crisis with oxygen and hydration, prevent crisis, screen for complications.

186
Q

What are the two classifications of AIHA (auto immune hemolytic anemia)?

A

Warm (IgG), and Cold (IgM)

187
Q

What diagnostic test is done for AIHA and what would be the result for both classifications (they’re different)?

A

Warm (IgG) would be positive for IgG, and warm (IgM) would be positive for complement.

188
Q

How are both types of AIHA treated?

A

1 (W+C). TREAT UNDERLYING CAUSE and warm patient (C)

2 (W+C). immunosuppression

  1. splenectomy (W), plasmapheresis (C)
189
Q

What is MAHA (microangiopathic hemolytic anemia)?

A

Hemolytic anemia due to intravascular fragmentation of RBCs.

190
Q

What are the major types of MAHA?

A

TTP/HUS, DIC, HEELP

191
Q

What would a blood peripheral for MAHA show?

A

Shistocytes (fragmented RBCs), evidence of hemolysis

192
Q

What is G6PD deficiency?

A

A deficiency in G6PD which leads to RBC sensitivity to oxidative stress

193
Q

How does someone get G6PD?

A

It is X-linked recessive. So it is inherited.

194
Q

What is the treatment for G6PD?

A
  1. Avoid offending stressers/triggers!!
  2. transfusion in SEVERE cases
195
Q

What is another name for vitamin B12?

A

Cobalamin

196
Q

What type of anemia does vitamin B12 deficiency result in?

A

Macrocytic

197
Q

Where is vitamin B12 absorbed in the GI and what types of problems may result in a B12 deficiency?

A

Terminal ileum.

Diet (strict vegan), Gastric/Intestinal Absorption (lack of IF, gastrectomy, pernicious anemia, malabsorption)

198
Q

What is the pathophysiology of pernicious anemia and what age group is it most common in?

A

Auto-antibodies produced against gastric parietal cells leading to lack of IF (intrinsic factor).

(May be associated to other autoimmune disorders)

Usually older > 60 y.

199
Q

What are the investigations for B12 deficiency anemia?

A

CBC, blood film, retic count, serum B12 and RBC folate (be aware that low B12 leads to low RBC folate)

200
Q

How is B12 deficiency anemia treated?

A

B12 IM monthyl for life or PO daily if intestinal absorp intact

**watch for hypokalemia and rebound thrombocytosis

201
Q

Body stores are good for how long for B12 and folate (each is different)?

A

B12 3-4 yrs, folate 3-6 months

202
Q

What are clinical features of B12 or folate deficiencies?

A

mild jaundice, glossitis, neurologic problems (B12 only!!!)

203
Q

How is folate deficiency diagnosed?

A

Rule out B12 first via serum B12 and than RBC folate.

204
Q

What are the three phases of hemostasis?

A
  1. primary (platelets)
  2. secondary (coagulation cascade)
  3. fibrin stabilization and eventual dissolution
205
Q

What is ITP?

A

Immune thrombocytopenic purpura – autoimmune disorder targeting platelets.

206
Q

How is ITP diagnosed?

A

Diagnosis of exclusion: Isolated thrombocytopenia

207
Q

What are clinical presentations of ITP?

A

Usually none! Minimal bruising but may result in serious bleed following trauma.

208
Q

What would PT and PTT show in ITP?

A

NORMAL

209
Q

What are the treatments for ITP?

A

corticosteroids or IVIG (first line), splenectomy (second line)

210
Q

What vaccinations does one need prior to splenectomy?

A

H. influenza, N. meningitis, Strep. pneumo

211
Q

What is HIT?

A

Heparin-induce thrombocytopenia (anti-body to heparin-platelet combination)

212
Q

What is TTP (thrombotic thrombocytopenic purpura)?

A

A MAHA caused by the spontaneous aggregation of platelets and activation of coagulation in small blood vessels.

213
Q

What are the clinical features of TTP?

A

THROMBOCYTOPENIA, MAHA (shistocytes), neurological symptoms (headache, confusion, seizures), renal failure, fever

214
Q

What is HUS (hemolytic uremic syndrome)?

A

A MAHA caused by activation of platelet aggregation/coagulation as a result of damage caused to vessel walls by a toxin (usually E. coli 157:7).

215
Q

What are the clinical features of HUS?

A

THROMBOCYTOPENIA, renal failure, MAHA (shistocytes)

216
Q

How are TTP and HUS investigated?

A

CBC and blood film: decreased plates, shistocytes

PT, PTT, fibrinogen: N (unlike DIC)

inc. bili & LDH, dec. haptoglobin
- ve Coombs’

217
Q

How are TTP and HUS treated?

A

MEDICAL EMERGENCY

Plasmapheresis +/- steroids

NO PLATELET TRANSFUSION! (inc. thrombosis)

218
Q

What investigations are done for vWD (von Willebrand disease)?

A

PTT (increased), dec. VIII (5-50%, vWF acts as carrier), plate count normal, dec. ristocetin cofactor (normally causes vWF to bind plates), dec vW (unless qualitative defect)

219
Q

How is vWD treated?

A

desmopressin (DDAVP) - causes release of vWF and VIII

220
Q

How does someone get hemophilia?

A

Inherit it, X-linked recessive

221
Q

How are hemophilias investigated?

A

PTT (prolonged), normal INR

222
Q

How are the hemophilias treated?

A

recombinant factor VIII (hemo A), IX (hemo B)

223
Q

What coagulation factors are NOT made in the liver?

A

VIII and vW

224
Q

Why does liver disease affect primary hemostasis (platelets)?

A

Splenomegaly secondary to PHT causes 2ndary thrombocytopenia due to sequestration.

225
Q

How is secondary hemostasis (coag factors) affected by liver disease?

A

All, but VIII, made in liver! Hello!!!

226
Q

What coag factors and inhibitors are affected by vit K deficiency?

A

X, IX, VII, II, C, and S

227
Q

What is the mechanism of DIC?

A

Uncontrolled release of plasmin and thrombin leading to intravascular coagulation and develoption of plates, coag factors and fibrinogen.

228
Q

How is DIC investigated and what are the corresponding lab results?

A

Dec. plates, inc INR PTT, dec. fibrinogen

Fibrinolysis: inc. FDPs or D-dimers

229
Q

What is Virchow’s Triad of venous thromboembolism?

A
  1. endothelial damage
  2. venous stasis
  3. hypercoagulability
230
Q

How is VTE (venous thromboembolism) treated?

A

Initial: anticoagulant (e.g. UFH, LMWH, or alternatives to)

Long-term: warfarin, IVC filters

231
Q

What is factor V leiden?

A

Factor Va cannot be inactived by activated protein C.

232
Q

What are the clinical features of AML?

A

UNCONTROLLED GROWTH OF BLASTS

anemia

thrombocytopenia

neutropenia

skeletal pain

233
Q

What are the investigations for AML?

A

CBC

INR, PTT, FDP, fibrinogen (in case of DIC)

peripheral blood film w/ AUER RODS

Bone marrow asp/bio

234
Q

How is AML treated?

A

Chemo, possibly stem cell xplant, G-CSF if inc risk of infection

235
Q

What are the 3 phases of CML?

A
  1. chronic phase (most diagnosed here)
  2. accelerated phase (inc. circulating blasts)
  3. blast crisis (transformation into AML/ALL)
236
Q

What are the clinical presentations of CML?

A

Mostly asymptomatic (incidental finding), +/- B-symptoms, splenomegaly, early satiety (enlarged spleen), anemia, bleeding (platelet dysfunction), pruritus

237
Q

How is CML investigated?

A

CBC w/ diff (neutrophilia, basophilia, eosinophilia), left shift and disorderly, anemia

bone marrow asp/bio inc. megakaryocytes

cytogenetics for t9;22 Phil chr

abdo imaging for spleen

238
Q

How is CML treated?

A

Chronic phase: imatnib mesylate (Gleevec)

bone marrow transplant if progression to accel. or blast phase (curative)

stem cell xplant for younger patients (curative)

239
Q

What is essential thrombocytosis?

A

Overproduction of platelets in the absence of recognizable stimulus.

240
Q

How is ET diagnosed?

A

MUST MEET ALL 4:

  1. sustained plate count > 450
  2. bone marrow bio show prolif megakar. (no change to granulocytes)
  3. not PV, CML, MDS or other myeloid neo
  4. no evidence of reactive thrombocytosis
241
Q

What are clinical features of ET?

A

often asymptomatic, thrombosis, bleeding (plate > 1000), splenomegaly

242
Q

How is ET investigated?

A

CBC, bone marrow asp/bio, excluse other MPN or reactive thrombo

243
Q

How is ET treated?

A

observation if asymptomatic, low dose ASA if thrombo event Hx or risk

244
Q

What is CLL?

A

Indolent disease characterized by clonal malignancy of mature B-cells.

245
Q

What are the clinical features of CLL?

A

Lymphadenopathy, splenomegaly, hepatomegaly, marrow failure (late stage)

246
Q

How is CLL investigated and what would test results show?

A

CBC (lymphocytosis), peripheral (small/mature lymphocytes, smudge cells), flow cytometry, cytogenetics, BM asp

247
Q

How is CLL treated?

A

CANNOT BE CURED

Observation if early

Intermittent chemo

Cortico or IVIG for autoimmune complications

Radiotherapy

248
Q

What does AHTR (acute hemolytic transfusion reaction) occur as a result of?

A

ABO incompatibility.

249
Q

What is FNHTR (febrile non-hemolytic transfusion reaction) caused by?

A

Due to alloantibodies to WBC, platelets or other donor plasma antigens.

250
Q

What is ALL?

A

Malignant disease of BM in which early lymphoid precursors (blasts) proliferate and replace normal hematopoietic cells of marrow.

251
Q

What are clinical features of ALL?

A

Young children

bone pain, anemia, neutropenia, thrombocytopenia (infiltration)

Lymphadenopathy

Hepatosplenomegaly

252
Q

How is ALL treated?

A

Eliminate abnormal clone via chemo (induction, consolidation, maintenance, prophylaxis)

253
Q

What is Hodgin Lymphoma?

A

Malignant proliferation of lymphoid cells with Reed-Sternberg cells (thought to arise from germinal centre B-cells).

254
Q

What type of infection has a strong correlation with Hodgkin Lymphoma?

A

EBV (mono)

255
Q

What are the clincal features of Hodgkin lymphoma?

A

Asymptomatic lymphadenopathy

Hepatosplenomegaly

Mediastinal mass

B-symptoms

Orderly spread from node-to-node

256
Q

How is Hodgkin lymphoma investigated/diagnosed?

A

Excisional lymph node biopsy

257
Q

How is Hodgkin treated?

A

Chemo, radiotherapy and if relapse occurs more chemo and BM xplant

258
Q

What is NHL?

A

Malignant proliferation of lymphoid cells of progenitor or mature B or T cells.

259
Q

What are the 3 categories/classifications of NHL?

A
  1. indolent - e.g. follicular, CLL
  2. aggressive - e.g. diffuse large B-cell
  3. highly aggressive - Burkitt’s
260
Q

How is NHL treated?

A
  1. indolent - symptom management and watchful waiting, radiation thx for localized disease
  2. aggressive - goal curative, chemo, radiation, relapse BMT
  3. highly aggressive - chemo
261
Q

Rai Staging of CLL – there are 5 steps what are they?

A

0 - lymphocytosis

1 - lymphadenopathy

2 - hepatosplenomegaly

3 - anemia

4 - thrombocytopenia

MDC01 (7 decks)

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