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Objectives:

To understand what is Myelophthisic Anemia is all about based on its


discovery
To classify based on etiology and morphology
To be able to know the mechanism or pathogenesis of the
Myelophthisic Anemia
To be familiarize with its signs and symptoms
To be able to know how to identify its screening and confirmatory tests
To discover treatments and prevention regarding anemia of chronic
disease

A. Intoduction
Myelophthisic anemia is a common finding in patients with carcinoma.
It is most often a hypoproliferative anemia classified as the anemia of
chronic inflammation (chronic disease). In some patients the degree of
anemia correlates with the tumor burden. In others, however, there is no
good correlation between tumor burden and the degree of anemia.
Myelophthisic anemia is caused by space occupying disorders of the
bone marrow. The bone marrow becomes infiltrated with fibrotic,
granulomatous or neoplastic cells. It is also the infiltration of abnormal cells
into the bone marrow and subsequent destruction and replacement of the
normal hematopoietic cells. It is a form of bone marrow failure that results
from the destruction of bone marrow precursor cells and their stroma, which
nurture these cells to maturation and differentiation. It is a type of bone
marrow failure caused by the invasion or replacement of normal bone
marrow tissue by abnormal tissue.
B. Etiology and Morphology
Three main causes of Myelophthisic Anemia:
1) metastatic cancer (most common)
2) infiltrative process (granulomatous diseases)
3) spent phase of myeloproliferative disorders
Other causes of this anemia are the following:

Macrophage proliferation in storage diseases


a) Gauchers Disease
b) Niemann-Pick disease

c) Schuller-Christian disease
Agnogenic myeloid metaplasia
Myelofibrosis
Leukemia
In terms of morphology of the cell of a person with this anemia, is
usually normocytic (MCV = 80-100) and normochromic (27-33 pg) which
results to the normal size and hemoglobin content of the cell.
C. Pathogenesis
Marrow fibrosis causes abnormal release of nucleated erythroid precursors
and immature granulocytic. Production of cytokines such as fibroblast growth factor
by the abnormal hematopoietic cell clone (particularly by megakaryocytes) leads to
replacement of the hematopoietic tissue of the bone marrow by connective tissue
via collagen fibrosis. The decrease in hematopoietic tissue impairs the patient's
ability to generate new blood cells, resulting in progressive pancytopenia, a
shortage of all blood cell types. However, the proliferation of fibroblasts and
deposition of collagen is a secondary phenomenon, and the fibroblasts themselves
are not part of the abnormal cell clone.
D. Definitive Signs and symptoms
Person with Myelophthisic anemia experiences the following signs and symptoms:

Fatigue
Malaise
Tachycardia
Chest pain
Shortness of breath
Splenomegaly
Bruising and bleeding under the skin (petechiae)
Paleness (pallor) of skin and fingernail beds

E. Screening and confirmatory test used to determine the type of anemia


Screening test includes:
Routine CBC Test
- if CBC findings shows normocytic, normochromic anemia with decreased
RBC and normal to decreased WBC count.
- presence of reticulocyte shows decreased amount
Peripheral smear
- It shows RBC indices and presence of anisocytosis, poikilocytosis and
polychromasia can be seen in blood
Confirmatory test:

Bone marrow examination or bone marrow biopsy


Bone Marrow Trephine biopsy (required)
Bone marrow examination refers to the pathologic analysis of samples of
bone marrow obtained by bone marrow biopsy (often called a trephine biopsy) and
bone marrow aspiration. Bone marrow examination is used in the diagnosis of a
number of conditions, including leukemia, multiple myeloma, lymphoma, anemia,
and pancytopenia. The bone marrow produces the cellular elements of the blood,
including platelets, red blood cells and white blood cells. While much information
can be gleaned by testing the blood itself (drawn from a vein by phlebotomy), it is
sometimes necessary to examine the source of the blood cells in the bone marrow
to obtain more information on hematopoiesis; this is the role of bone marrow biopsy.
F. Blood picture

Peripheral blood film at


1000X magnification

This blood film at 1000X


magnification demonstrates a
leukoerythroblastic blood picture
with the presence of precursor cells
of the myeloid and erythroid
lineage. In addition, anisocytosis,
poikilocytosis, and polychromasia
can be seen.

Bone marrow film at


400X magnification

This bone marrow film at 400X


magnification demonstrates
carcinoma metastasis. Bone
marrow cells are completely
replaced by large carcinoma cells
with clear nucleoli.

Treatment:

Treat the underlying disease and provide supportive measures for


symptomatic patient
Treat anemia with packed red blood cell transfusions
Patients with low erythropoietin levels and may respond to supplemental
erythropoietin therapy

G. Case Study
A 66-year-old man presents with history of back and chest pain, fatigue and
experiences the sensation of a rapid or intense heartbeat. Examination of his
peripheral blood smear reveals the abnormal presence of nucleated red blood cells,
immature white blood cells, myelocytes and immature granulocytes.
His CBC result:
Hb = 9.3 g/dL
Hct = 27.4%
MCV = 88fL
MCH =28 pg
Reticulocyte count = 1.8%

Sources:

1. Rodak, B, F., Fritsma, G, A., Keohane, E,M., Hematology: Clinical


Principles and Application. 4th ed. 2012. Elsevier Saunders Inc.
Missouri, USA.
2. Lotspeich-Steininger, C.A., Stiene-Martin, E.A., & Koepke, J.A. Clinical
Hematology: Principles, Procedures, Correlations.1992. J.B.
Lippincott Company. Philadelphia, P,A, USA.
3. Hart, M, N,. Loeffler, A, G,. Human Diseases: Pathophysiology for
Health Proffesionals. Wisconsin, Madison.

4. Gupta, A., Nayak, R,. Rai, S,. Essentials in Hematology and Clinical
Pathology.

5. Besa, E,C., Krishnan, K., Talavera, F. Myelophthisic Anemia. USA.


6. Lichthin, A,E,. MSD Manual Profession Edition. 2013. USA.
7. Goldman: Cecil Medicine, 23rd ed., Saunders 2007: Chapter 162
Approach to the Anemias.
8. Proforma Inc. (2010). Retrieved from http://webhealth.com/anemia/rbcproduction/myelophthisic/
9. Makoni, S. N,. Encylopedia of Molecular Mechanisms of Disease.
(2009). pp 1404-1405.
Berlin.

MT 2a : Hematology 1

Myelophthisic Anemia

Chinny Marie C. Palabrica


MLS 3-C

Mennen Ace Q. Laude, RMT


Instructor