dysmyelopoietic disorders

Question 1

a condition where a new pluripotential stem cell appears in the marrow

Answer 1

DYSMYELOPOIETIC SYNDROMES (DMPS)

Question 2

The process is different from the CMP disorders

Answer 2

(DMPS)
(-) OVERPRODUCTION
(+) DYSPLASIA of the myeloid cell lines

Question 3

(DMPS) Often characterized by ___ either an

Answer 3

PERIPHERAL CYTOPENIAS secondary to

• -ABSOLUTE DECREASE IN PRECURSOR CELLS in the marrow or
• -INEFFECTIVE CELL PRODUCTION

Question 4

TWO HYPOTHESES

Answer 4

Represents the growth of an abnormal clone of cells in the marrow

Abnormal DMPS stem cell line has the ability to differentiate to mature end stage cells that are abnormal in appearance and function

Question 5

(DMPS) Triad of symptoms

Answer 5

• Fatigue
• Fever – due to infection secondary to granulocytopenia
• Bleeding – due to thrombocytopenia
• PALLOR may be present secondary to anemia
• The spleen may be slightly enlarged but there is no lymphadenopathy

Question 6

PERIPHERAL AND BONE MARROW ABNORMALITIESERYTHROPOIESIS

• May show quantitative abnormalities that may range from
• The hallmark is

Answer 6

-RED CELL APLASIA to extreme ERYTHROID HYPERPLASIA
-DYSERYTHROPOIESIS
-Megaloblastoid maturation
-Nuclear irregularity
-Multinuclearity may be present
-Left shift
-Hemoglobinization of the cytoplasm (hypochromic microcytic)
Ringed Sideroblasts

Question 7

MEGALOBLASTOID vs MEGALOBLASTIC

Nuclear chromatin

Answer 7

Coarser clumping==Delicate

Question 8

MEGALOBLASTOID vs MEGALOBLASTIC

Morphologies

Answer 8

Macrocytic anemia with oval macrocytes, basophilic stippling, Howell-Jolly bodies, nucleated red cells, and giant platelets with decreased platelet granulation

Question 9

MEGALOBLASTOID vs MEGALOBLASTIC

Vitamin B12/Folate levels

Answer 9

Normal==Decreased

Question 10

MEGALOBLASTOID vs MEGALOBLASTIC

Other myeloid cell lines

Answer 10

Affected==Not affected

Question 11

MEGALOBLASTOID vs MEGALOBLASTIC
Neutrophilic hypersegmentation
Micromegakaryocytes
Megakaryoblasts

Answer 11

+ +
+ -
+ -

Question 12

PERIPHERAL AND BONE MARROW ABNORMALITIESGRANULOPOIESIS

Answer 12

• -Decreased granulopoiesis to marked granulocytic hyperplasia
• -Dysgranulopoiesis is manifested by a left shift with increase in myeloblasts
• -Hyposegmentation with pseudonucleation (Pelger-Huet forms)
• -Hypogranulation or coarse abnormality in granulation (pseudo Chediak-Higashi) – rarely in eosinophils and basophils
• -Neutrophils with monocytoid features

Question 13

TYPE I BLASTS

Answer 13

• Myeloblasts with a
• *centrally located nucleus, a
• *fine nuclear chromatin pattern and a
• *prominent nucleolus
• Cytoplasm contains **no granules

Question 14

TYPE II BLASTS

Answer 14

• Myeloblasts with
• *centrally located nucleus but
• *slightly more mature nuclear chromatin pattern and a
• *lower N:C ratio
• Large granules** in cytoplasm

Question 15

PERIPHERAL AND BONE MARROW ABNORMALITIESMEGAKARYOPOIESIS

Answer 15

Near absence or hyperplasia
Most common : Maturation arrest
Thrombocytopenia

Question 16

SPECIFIC CLINICOPATHOLOGIC FORMS OF DYSMYELOPOIETIC SYNDROMES (FAB)

Answer 16

(RA/RC)
(RARS)
–Idiopathic Acquired Sideroblastic Leukemia
(RAEB)
(CMML)
–Ph1-Negative Chronic Granulocytic Leukemia or Juvenile CGL
(RAEBIT)

Question 17

Acute Leukemia LABORATORY TESTS IN DIAGNOSIS

Answer 17

Complete blood count
Blood film examination
Bone marrow aspirate/Biopsy

Others:
Vitamin B12/Folate
Serum Iron and Ferritin
Iron stains (Prussian blue)
Reticulin stain (r/o myelofibrosis)
Peroxidase stain (r/i Auer rods)
Esterase stain (% of monocytic cells)

Cytogenetics
Lead and arsenic levels
Stem cell culture
Flow cytolometry (DNA studies)