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Heme > Bone Marrow Disorders > Flashcards

Bone Marrow Disorders Flashcards

1
Q

What is Pure Red cell Aplasia (PRCA)?

-aka

A
  • erythroblastopenia
  • type of anemia affecting the precursors to red blood cells .
  • severe reduction in the reticulocytes in peripheral blood
  • virtual absence of erythroid precursors in bone marrow
  • bone marrow often ceases to produce RBC
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2
Q

Causes of Pure Red Cell Aplasia?

A
  • autoimmune disease
  • Thymoma
  • Viral infections such as HIV, herpes, parvovirus B19
  • Large granular lymphocyte leukemia
  • Idiopathic
  • Congenital: “hereditary pure red cell aplasia” has been used to refer to Diamond Blackfan anemia
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3
Q

Pure Red Cell Aplasia Dx is suspected in a pt with:?

It is confirmed with?

A
  • Dx is suspected in a pt with:
  • anemia and very low reticulocyte count
-confirmed with: 
normocytic normochromic anemia 
-normal WBC and platelets
-normocellular bone marrow
-reticulocyte count
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4
Q

Pure red cell aplasia Tx

A
  • red cell transfusion for symptomatic anemia
  • cessation of possible drugs causing this disease
  • immediate hematologic consultation
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5
Q

What is the prognosis of Pure Red Cell Aplasia?

A
  • remitting and relapsing course requiring long-term support of their anemia with red cell transfusions
  • long term tx with immunosuppressive or cytotoxic agents
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6
Q

Myelophthisic Anemia, what is this?

A
  • severe kind of anemia found in ppl w/ diseases that affect the bone marrow.
  • the displacement of hemopoietic bone-marrow tissue into the peripheral blood either by fibrosis, tumors, or granulomas.
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7
Q

What causes Myelophthisic Anemia?

A
  • chronic myeloproliferative diseases (myelofibrosis)
  • leukemia
  • lymphoma
  • Metastatic carcinoma or myeloma (breast, lung, or prostate cancer that has metastasized to bone marrow)
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8
Q

How do you diagnose Myelophthisic Anemia?

A
  • otherwise unexplained quantitative changes in one or more of the blood and bone marrow elements.
  • Blood smear; erythrocytes that contain nuclei or dacryocytes (tear drop cell)
  • leukoerythroblastosis; the displaced hematopoietic cells begin to undergo extramedullary hematopoiesis
  • blasts account for less than 20% of total cells of bone marrow aspirate and peripheral blood, cases with higher blasts are considered to have acute myeloid leukemia

*** DIagnosis confirmed when bone marrow biospy demonstrates significant replacement of bone marrow by fibrosis, malignancy, or other infiltrative process.

***MDS is a diagnosis of exclusions

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9
Q

Myelophthisic Anemia Tx?

A

-treat the underlying cause/cancer

  • consider bone marrow transplant in young
  • supportive (RBC transfusions, treat infections)
  • Growth Factors (EPO)
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10
Q

Myelodysplastic Syndrome (MDS) what is this?

A

-variable reduction in the production of normal red blood cells, platelets, and mature ganulocytes(neutro, eosino, baso)

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11
Q

Myelodysplastic Syndrome leads to what type of systemic consequences?

A
  • anemia
  • bleeding
  • increased risk of infection
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12
Q

What causes Myelodysplastic Syndrome?

A
  • most cases are idiopathic
  • environmental factors (chemicals, radiation, tobacco, chemo drugs)
  • Gene abnormalities (trisomy 21, blooms syndrome, Fanconis Anemia)
  • other hematologic diseases (paroxysmal nocturnal hemoglobinuria, congenital neutropenia)
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13
Q

40% of the time what does Myelodysplastic syndrome result in?

A

-acute leukemia

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14
Q

Clinical and laboratory manifestations of Myelodysplastic Syndrome?

A

-Anemia: macro, normocytic, oval macrocytes on peripheral smear

  • neutropenia
  • thrombocytopenia
  • purpura: bruising w/ thin skin in old age in sun exposed areas.
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15
Q

What is the only potentially curative therapy for MDS patients?

A

bone marrow therapy

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16
Q

What are some indicators of a good prognosis of MDS? Indicators of poor prognosis?

A
  • younger age
  • normal or moderately reduced neutrophil or platelet count
  • low blast counts in bone marrow
  • no auer rods (red staining needle like bodies seen in cytoplasm of myeloblasts)
  • advanced age
  • severe neutropenia, thrombocytopenia
  • high blast count
  • auer rods
17
Q

Stem Cell definition

A
  • a cell that has the ability to continuously divide and differentiate into various other kind(s) of cells/tissues
18
Q

Kinds of Stem cells

A
  • embryonic stem cells: come from 5-6 day old embryo. can form virtually any type of cell in the body.
  • embryonic germ cells: derived from part of a human embryo or fetus that will ultimately produce eggs or sperm
  • adult stem cells: undifferentiated cells found among specialized or differentiated cells in a tissue or organ after birth. have a more restricted ability to produce different cell types and to self-renew
19
Q

Why are Hematopoietic Stem cell transplants performed?

Where are hematopoietic stem cells retrieved from?

A
  • to re-establish hematopoietic function in patients w/ damaged/defective bone marrow or immune systems.
  • potentially curative for a wide variety of disorders.
  • collected from the bone marrow, peripheral blood, or umbilical cord blood.
20
Q

What are the types of grafts?

A
  • allogenic: from another person
  • syngeneic: from identical twin
  • autologous: from the patient
  • umbilical cord blood
21
Q

Consequences of Autologous Transplant?

-whats one requirement you must meet to have autologous transplant?

A
  • transplant related mortality is lowest
  • relapse rates are higher depending on disease
  • absence of graft versus host effects

Requirement:
-no evidence of disease in the blood or bone marrow

22
Q

Consequences of Allogenic transplant?

A
  • High transplant mortality
  • lower relapse rate due to graft vs tumor effects
  • graft vs host effects (transplant attacks host cells)
23
Q

What is the chance (%) of having a sibling match for a graft?

A

25%

24
Q

Consequences of Matched unrelated donors in transplant?

A
  • severe graft vs host disease

- Higher transplant related mortality

25
Q

Who are considered haploidentical donors?

A

-from parent, child, or sibling

26
Q

Umbilical cord blood:

how is this stored? How is cell dose given?

A
  • obtained from umbilical vein and frozen with an anticoagulant and nutrient media.
  • per recipient weight
27
Q

How are stem cells collected?

A
  • Bone marrow harvest: general anesthesia
  • 2 deaths in 8000 collections?
  • stem cells circulate in the blood, identified by CD34+ by flow cytometry, colleced through apheresis catheter.
  • higher GVHD than bone marrow harvest
  • more rapid marrow recovery
28
Q

How long can we wait before infusing stem cells?

A

may be used fresh within a few hours of collection or frozen using DMSO

29
Q

Early Complications of Stem Cell/graft

A
  • mucositis (painful inflamm and ulceration of mucous membranes lining the digestive tract)
  • sinusoidal obstructive syndrome (VOD; veno-occlusive disease) fluid retention, jaundice, hepatomegaly
  • transplant related infections– damage to mouth, gut, skin, prolonged neutropenia, pnacytopenia (deficiency of RBC, WBC, and platelets)
  • Graft vs host Disease, graft rejection, interstitial pneumonitis
30
Q

Delayed complications of stem cell/graft ?

A
  • Chronic GVHD
  • secondary tumors (Acute leukemias)
  • late infections: bacterial, viral, fungal

Heme (13 decks)

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