WHO classification and criteria for the myelodysplastic syndromes (MDS)


Refractory Anemia (RA)

Peripheral Blood

Anemia

No or rare blasts

Bone Marrow

Erythroid dysplasia only

<5 percent blasts

 <15 percent ringed sideroblasts


Refractory Anemia with Ringed Sideroblasts (RARS)

Peripheral Blood

Anemia

No blasts

Bone Marrow

Erythroid dysplasia only

<5 percent blasts

 
15 percent ringed sideroblasts


Refractory Cytopenia with Multilineage Dysplasia (RCMD)

Peripheral Blood

Bi- or pan-cytopenia

No or rare blasts

No Auer rods

Monocytes <1,000/µL

Bone Marrow

Dysplasia in
10 percent of cells in two or more myeloid cell lines

No Auer rods

<5 percent blasts

<15 percent ringed sideroblasts


Refractory Cytopenia with Multilineage Dysplasia and Ringed Sideroblasts (RCMD-RS)

Peripheral Blood

Bi- or pan-cytopenia

No or rare blasts

No Auer rods

Monocytes <1,000/µL

Bone Marrow

Dysplasia in
10 percent of cells in two or more myeloid cell lines

No Auer rods

<5 percent blasts

15% ringed sideroblasts


Refractory Anemia with Excess Blasts-1 (RAEB-1)

Peripheral Blood

Cytopenias

<5% blasts

No Auer rods

Monocytes <1,000/µL


Bone Marrow

Unilineage or multilineage dysplasia

5-9 percent blasts

No Auer rods


 

Refractory Anemia with Excess Blasts-2 (RAEB-2)

Peripheral Blood

Cytopenias

5-19% blasts

Auer rods ±

Monocytes <1,000/µL

Bone Marrow

Unilineage or multilineage dysplasia

10-19 percent blasts

Auer rods ±


MDS-Unclassified (MDS-U)

Peripheral Blood

Cytopenias

No or rare blasts

No Auer rods

Bone Marrow

Unilineage dysplasia in granulocytes or megakaryocytes

No Auer rods

<5 percent blasts


MDS with del(5q) "5q- syndrome"

Peripheral Blood

Anemia

<5 percent blasts

Platelets usually normal or increased

Bone Marrow

Normal to increased megakaryocytes with hypolobulated nuclei

No Auer rods

<5 percent blasts Isolated del(5q)


Note: In this proposed WHO system, the FAB MDS subgroup Refractory Anemia with Excess Blasts in Transformation (RAEB-T) has been taken out of the MDS classification and is now considered Acute Myeloid

Leukemia with Multilineage Dysplasia.


Adapted from Brunning, RD, et al. Myelodysplastic syndromes: Introduction. In: Jaffe, ES, Harris, NL, Stein, H, Vardiman, JW, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. IARC Press: Lyon 2001, p. 63. Permission granted from Harris, NL and Vardiman, JW.

 

Subtype incidence — Partitioning of 1640 cases taken from the available literature according to the original FAB classification system was:

•Refractory anemia — 21 percent

•Refractory anemia with ringed sideroblasts — 17 percent

•Refractory anemia with excess blasts — 37 percent

•Refractory anemia with excess blasts in transformation — 12 percent

•Chronic myelomonocytic leukemia — 13 percent


TREATMENT GUIDELINES from Up to Date


I. Classification

III. Treatment

Calculator: Myelodysplastic Syndrome International Prognostic Scoring System


Percentage of Bone Marrow Blasts


< 5 percent (0 points)

5 to 10 percent (0.5 points)

11 to 20 percent (1.5 points)

21 to 30 percent (2.0 points)


Karyotype


Normal, Y-, 5q-, 20q- (0 points)

Abnormal chromosome 7 or 3 or more abnormalities (1.0 points)

All other cytogenic abnormalities (0.5 points)


Cytopenias

(defined as hemoglobin < 10 g/dL, absolute neutrophil count < 1800/microL, platelet count < 100,000/microL)


No cytopenia or cytopenia of 1 cell type (0 points)

Cytopenia of 2 or 3 cell types (0.5 points)


Total Criteria Point Count:

Prognostic Score Interpretation

0 Points : Low (Median Survival of 5.7 yrs)                                                                                                 0.5 - 1 Points : Intermediate 1 (Median Survival of 3.5 yrs.)                                                                          1.5 - 2.0 Points : Intermediate 2 (Median Survival of 1.2 yrs.)                                                            2.5-3.5 Points : High (Median Survival of 0.4 yrs.) 



Median survival (in years) in myelodysplastic syndrome according to IPSS and age


IPSS risk group                      Age
60    Age >60    Age >70

Low                                            11.8            4.8            3.9

Intermediate-1                            5.2              2.7            2.4

Intermediate-2                            1.8              1.1            1.2

High                                           0.3              0.5            0.4


Adapted from Greenberg, P, Cox, C, Le Beau, MM, et al, Blood 1997; 89:2079.

 
II. Prognosis
I. Classification II. Prognosis III. Treatment

                            EPO use Guidelines – Oct 2009


For Chemotherapy Induced Anemia


I. Recommended to obtain lab work each time drug is administered for reimbursement.


II. Medicare Guidelines – ESAs are not indicated for patients receiving  myelosuppressive therapy when the anticipated outcome is cure.


  1. III.For chemotherapy and radiation induced anemia: Hgb must be less than 10.0 or  Hct less than 30.0.


ICD-9 Codes for Chemotherapy induced anemia (3 code combo):

285.9      Anemia

995.20    Adverse Effect medicinal substance

E933.1   Antineoplastic and/or immunosuppressive drugs


IV. Maximum dose 50,000 units weekly.


And for radiotherapy-induced anemia

Hgb must be less than 10.0 or Hct less than 30.0.      


ICD-9 Codes for Radio-induced anemia (2 code combo):

285.9     Anemia

E879.2   Adverse effect radiation

   

IV. Also maximum dose is 50,000 units weekly.


For non-chemotherapy or radiation induced anemia

For documentation of Myeodysplastic S. and Anemia requiring treatment it is needed to document 2 codes, one for MDS and one for anemia.

Example:

Assessment and Plan

  1. 1.Myelodysplastic Syndrome (238.72)

  2. 2.Anemia (285.9) due to Myelodysplastic Syndrome. Proceed with Procrit treatment and instruction as specified on Procrit order sheet.


Codes for use with


  1. 1.Chronic Renal Insufficiency (2 code combo):

285.21  Anemia in chronic kidney disease AND

585.9    Renal insufficiency

  1. 2. Myelodysplasia (2 code combo):

238.72  Myelodysplastic Syndrome (low grade) OR

238.73 MDS High Grade, OR

238.74 MDS with 5q deletion, OR

238.75 MDS unspecified, AND

285.9    Anemia

  1. 3.Hepatitis C (2 code combo):

285.29   Anemia of Chronic Disease AND

070.70   Hepatitis C

  1. 4.HIV (2 code combo):

285.29   Anemia of Chronic Disease  AND

V08       HIV status asymptomatic

  1. 5.Rheumatoid Arthritis ( 2 code combo):

285.29  Anemia of Chronic Disease  AND

714.0    Rheumatoid Arthritis

  1. 6.Pre-surgical

V72.85  Spec Pre-Operative Exam  AND

285.9     Anemia, unspecified.



  3. I. Initial pretreatment Hgb must be less than 10.0 or Hct less than 30.0              


II. To continue ESA, Hgb must be less than 11.0 or Hct less than 36.0.


III. Notice that maximum dose is 90,000 units weekly.


Helps with EPO Dictation


Most always dictate Anemia alone, as a diagnosis (and code) when using  EPO.


Always put diagnosis that we need for coding in ending of dictation (assessment and plan section).


Be definitive, don’t say “may be due to”, “seems to be”.


If Macrocytic Anemia is dictated, EPO will not be covered.


If iron deficiency is dictated, EPO will not be covered.


If B12 deficiency is dictated, EPO will not be covered


Sideroblastic Anemia is not covered, Sideroblastic Anemia, Refractory is covered as MDS low grade.