Anemia - Ministry of Public Health Thalassemia Major (Cooley anemia) moderate to severe anemia [Hb

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Chapter III D45-D89 Diseases of the blood and blood-forming organs นพ.บก เจญล กมงานลยกรรม รพ.สวรรประชาก นครสวรร 1 Documentation/Coding of Anemia Looking for Specific Documentation Type of anemia [i.e. Acute blood loss, Aplastic anemia, Pure red cell aplasia, Hemolytic anemia, Anemia in chronic disease] Review Laboratory/Pathology findings Blood values following a surgical procedure Significant drop of Hb/Hct Query the Physician for Specific diagnosis relating to these findings Underling cause of the anemia [e.g. long-term anticoagulants, gastric ulcer, neoplasms, internal bleeding, thalassemia] 2 Complete Blood Count Hemoglobin (g/dL) amount of oxygen carrying protein Hematocrit (%) % of blood volume occupied by RBCs RBC count (/mL) number of RBCs MCV (fL) mean corpuscular (cell) volume MCH (pg) mean corpuscular (cell) haemoglobin MCHC (g/dL) mean corpuscular (cell) haemoglobin concentration RDW (%) red cells distribution width WBC count (/mL) number of WBCs Platelet count (/mL) number of platelets Abnormal any Values are Not Disease, the Etiology is….. 3 Iron deficiency anemia Anemia from acute blood loss Anemia from chronic blood loss Anemia in chronic disease Anemia in neoplastic disease Hemolytic anemia Aplastic anemia Thalassemia Anemia 4

Transcript of Anemia - Ministry of Public Health Thalassemia Major (Cooley anemia) moderate to severe anemia [Hb

Chapter III D45-D89 Diseases of the blood and

blood-forming organs

นพ.บก เจญล

กมงานลยกรรม รพ.สวรรประชาก นครสวรร

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Documentation/Coding of Anemia

Looking for Specific Documentation Type of anemia [i.e. Acute blood loss, Aplastic anemia, Pure red cell

aplasia, Hemolytic anemia, Anemia in chronic disease] !

Review Laboratory/Pathology findings

Blood values following a surgical procedure

Significant drop of Hb/Hct !

Query the Physician for Specific diagnosis relating to these findings

Underling cause of the anemia [e.g. long-term anticoagulants, gastric

ulcer, neoplasms, internal bleeding, thalassemia]

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Complete Blood Count

Hemoglobin (g/dL) amount of oxygen carrying protein

Hematocrit (%) % of blood volume occupied by RBCs

RBC count (/mL) number of RBCs

MCV (fL) mean corpuscular (cell) volume

MCH (pg) mean corpuscular (cell) haemoglobin

MCHC (g/dL) mean corpuscular (cell) haemoglobin

concentration

RDW (%) red cells distribution width

WBC count (/mL) number of WBCs

Platelet count (/mL) number of platelets

Abnormal any Values are Not Disease, the Etiology is…..

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Iron deficiency anemia

Anemia from acute blood loss

Anemia from chronic blood loss

Anemia in chronic disease

Anemia in neoplastic disease

Hemolytic anemia

Aplastic anemia

Thalassemia

Anemia

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Anemia

Age/Gender group Hb threshold (g/dL) Hct (%)

Men (>15 years old) 13.0 39

Woman, non-pregnant (15 years old) 12.0 36

Woman, pregnant 11.0 33

Teens (12-15 years old) 12.0 36

Children (5-12 years old) 11.5 34

Children (0.5-5 years old) 11.0 33

WHO’s Hemoglobin thresholds used to define anemia

Anemia is present when the haemoglobin level is

more than 2SD below the mean.

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Anemia

Based on Morphology [Volume]

Normocytic - MCV 80-100 fL Acute blood loss, Aplastic anemia, Pure red cell aplasia, Hemolytic

anemia, Anemia in chronic disease, Renal failure, Marrow infiltration !

Microcytic - MCV <80 fL Iron deficiency anemia, Anemia in chronic disease (late), Thalassemia !

Macrocytic - MCV >100 fL Folate or B12 deficiency anemia, Myelodysplastic syndrome, Chronic

liver disease, Hypothyroidism, Reticulocytosis

Size Does Not Answer All Questions

Be Aware of Mixed Anemias..!!

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Anemia

Based on Etiology [Pathophysiology]

Decreased Production (Nutritional Deficiency) Iron deficiency, Folic acid and B12 deficiency, Malnutrition !

Decreased Production (Defects in Bone Marrow) Aplastic anemia, Leukemia, Lymphoma, Myeloproliferative syndrome,

Anemia in chronic disease, Infections (TB, HIV, Parvovirus) !

Blood Loss Acute or blood loss, Chronic blood loss, Hemolysis !

Increased Destruction Hemolytic anemia, G6PD deficiency, Spherocytosis, Ellipocytosis,

Thalassemia, Sickle cell disease, Paroxymal nocturnal hemolysis, DIC, TTP

Anemia is actually a sign of a disease process

rather than a disease itself.

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Normocytic Anemia

Low Reticulocyte Count (<0.5%)

Inadequate Production

High Reticulocyte Count (

Increased Destruction

Hereditary pure red cell aplasia

Diamond-Blackfan syndrome

Acquired pure red cell aplasia

Infection/Inflammation

(ineffective EPO production)

Medications

Bone marrow damage

Coombs Negative

Acute blood loss

Microangiopathic haemolytic anemia

Membrane defects

Hemoglobinopathies

Enzyme defects

Coombs Positive

Autoimmune hemolytic anemi

MCV 80-100 fL, MCH >27 pg

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Reticulocyte Count

Reticular [mesh-like] network of ribosomal RNA

Fully mature within 2 days (maturation)

Low reticulocyte <2.5%: Decreased marrow production of RBC

High reticulocyte >2.5%: Increased destruction of RBC

Immature RBC, normal 1%

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Anemia

แนวทางการจยและกษาภาวะโลตจาง

1. นกการจยของแพท

2. ประและการตรวจางกาย

3. ผลตรวจทางองปการ

4. การกษา หอการตรวจเมเม

การสปเวชระเยนและการใรส ไสามารถสปและใรสชดของภาวะโลตจาง

โดยจารณาจากผลการตรวจเอด Complete Blood Count เาน

โดยไการนกำจยในเวชระเยนของแพท

an=absence, hemia=blood, anemia=without blood

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Anemia from Acute Blood Loss

เกณการจย

1. ประการเยเอดอางเยบพนดเจน

2. ผลตรวจเอดสบสนาด

3. หอไการใเอดไ

4. นกการจยของแพท

“Acute post-hemorrhagic anemia”

“Anemia from acute blood loss”

“Acute blood loss”

“Anemia from blood loss”

Acute post-hemorrhagic anemia

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วยอาเยนเนเอด องกองกระเพาะอาหารพบ acute

hemorrhagic gastritis ผลตรวจ Hct เน 35% และใ PRC 2 ต

PDx Acute hemorrhagic gastitis K29.0

SDx

Anemia from acute blood loss D62

Op

Esophagogastroduodenoscope

Pack red cell transfusion

45.13

99.04

EC

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วยรถมอเตอไซคำ ญญาณพปก รอยำชายโครงางาย เอกซเรคอมวเตอององพบ subcapsular

hematoma of spleen with hemoperitoneum เจาะเอดดตาม

Hct ลดลงจาก 40% เน 35%

PDx Splenic injury S36.00

SDx

Acute post-hemorrhagic anemia D62

Op

CT abdomen 88.01

EC มอเตอไซคำเอง V28.4

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Anemia Posthemorrhagic Anemia

ประการเยเอดอางเยบพนดเจน

ผลตรวจเอดพบ normochromic normocytic, MCV >80 fL

นกการจยของแพท

การกษาหอไการกษาโดยการใเอดไ

D62 Acute posthaemorrhagic anaemia Excludes: congenital anaemia from fetal blood loss ( P61.3 )

D63* Anaemia in chronic diseases classified elsewhere

œ

Anemia from acute blood loss

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Iron Deficiency Anemia

Common nutritional deficiency

Bleeding is a leading cause of iron deficiency anemia

Microcytic hypochromic anemia [low MCV, MCH & MCHC]

Iron studies

- Low serum iron

- High total iron binding capacity (TIBC, transferrin conc.]

- Low % transferrin saturation

- Low ferritin

- Decrease BM storage iron (hemosiderin)

Hypochromic anemia

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Iron Deficiency Anemia

ผลตรวจเอดพบ hypochromic microcytic, MCV <80 fL,

anisocytosis & poikilocytosis ไมาก

อาจสบสนวย serum iron ำ, TIBC ง, iron storage ำ

การกษาโดยการใเหกทดแทน

กรประการญเยเอดดเจน

ในกา Iron deficiency anemia due to chronic blood loss

กรไประการญเยเอด

ในกา Iron deficiency anemia

Hypochromic anemia

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Anemia in Chronic Disease

IFN, TNF, IL block iron transfer from macrophage store to RBC

Mild anemia resistant to iron therapy

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Anemia in Chronic Disease

Mild to moderate anemia

Mildly microcytic or Normocytic anemia

Etiology – Chronic immune activation

Chronic infections: TB, SBE, Osteomyelitis

Collagen vascular disease: RA, SLE

Endocrine deficiency: Hypothyroidism, Panhypopituitarism,

Hypoadrenalism

Visceral organ failure: Renal failure, Hepatic failure

Malignancy: BM metastasis, Leukemia, Lymphoma, Myeloma

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Anemia in Chronic Disease

โรคเองำใเดภาวะโลตจาง

✦ Chronic kidney disease stage 3–5

✦ Endocrine disease: Hypothyroidism [not…DM, DLP]

✦ Connective tissue disease: Rheumatoid

✦ Chronic infection: Tuberculosis, AIDs, Liver disease

ผลตรวจเอดพบ normochromic normocytic, MCV >80 fL

อาจนนวย serum ferritin ปก, TIBC ำ, iron storage ปก

การกษาวยการใเอด หอการกษาวย erythropoietin

หอการตรวจเมเม

Not record as Principle diagnosis

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Blood Loss from Procedure

เกณการจย

1. นกการเยเอดในรายงานการาด

2. ผลตรวจเอดสบสนาด

3. หอไการใเอดไ

4. นกการจยของแพท

“Operative bleeding”

“Postoperative bleeding”

“Postoperative anemia” Postoperative anemia = D64.9

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Anemia

Summary Diagnosis ICD-10

Iron deficiency anemia

Iron deficiency anemia due to chronic blood loss

Anemia from chronic blood loss

Anemia from blood loss

D50.9

D50.0

D50.0

D50.0

Anemia from acute blood loss

Acute posthemorrhagic anemia

D62

D62

Anemia in chronic disease

Anemia in neoplasm

D63.8*

D63.0*

Operative bleeding

Postoperative anemia due to acute blood loss

T81.0

D62

Most specific type as possible

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Anemia in Cancer Patient

Decreased Production Increased Destruction Blood Loss

Anemia of inflammation

Bone marrow infiltration

Chemotherapy

Chronic kidney disease

Nutritional deficiency

!

Hemolysis Acute blood loss

Chronic blood loss

Cancer-related anemia (CRA)

CRA (non-chemotherapy-related)

Chemotherapy-induced anemia

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Anemia in Neoplastic Disease

มะเงงเนอ (active)

ผลตรวจเอดพบ normochromic normocytic, MCV >80 fL

อาจนนวย serum ferritin ปก

TIBC ปกหอ

serum/bone marrow ปกหอง

การกษาวยการใเอด หอการตรวจกษาเมเม

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Chemotherapy-Induced Anemia

Incidence increased from 19.5% in cycle #1 to 46.7% by cycle #5

Time to nadir hemoglobin level estimated at 2 weeks

Treatment: RBC, Erythropoietin stimulating agent (ESAs)

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Anemia in Cancer Patient

Summary Diagnosis ICD-10

Anemia in neoplastic disease

Chemotherapy-induced anemia (CIA)

Chemotherapy-induced aplastic anemia

D63.0*

D60.1

D61.1

Antimetabolites

Natural products

Antineoplastic antibiotics

Miscellaneous

Y43.1

Y43.2

Y43.3

Y43.3

Note: Chemotherapy-induced anemia, ICD-10-CM = D64.81

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Anemia in Cancer PatientSummary Diagnosis ICD-10

!

Folic acid analogue: Methotrexate

Pyrimidine analogue: 5-FU, Capecitabine, TS-ONE,

Cytarabine, Gemcitabine

Purine analogue: Mercaptopurine

Y43.1

!

Vinca alkaloids

Taxanes

Epipodophyllotoxins

Camptothecins: Irinotecan, Topotecan

Y43.2

!

Thalidomide, AzathioprineY43.4

Antineoplastic Antimetabolites

Antineoplastic Natural Products

Immunosuppressive Agents

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Anemia in Cancer PatientSummary Diagnosis ICD-10

other Antineoplastic Drugs

Antineoplastic antibiotics: Actinomycin, Doxorubicin,

Bleomycin, Mitomycin C, Mitomycin, Mitoxantrone,

Asparaginase

Alkylating agents: Chlorambucil, Cyclophosphamide,

Ifosfamide, Busulfan, Streptozocin, Meclorethamine,

Melphalan, Nitrourea, ThioTEPA

Miscellanous: Cisplatin, Carboplatin, Hydroxyurea

Target agents

Rituximab, Trastuzumab, Cetuximab, Bevacizumab,

Gemtuzumab

Y43.3

Note: จารณาตามกมของยา (อาจางจาก Table of Drugs And Chemical)

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Thalassemia

Inherited autosomal recessive blood disorder

characterised by abnormal formation of haemoglobin

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3 Basic Types of Hemoglobin

HbA : 2 alpha + 2 beta chains forming a tetramer

97% of adult haemoglobin

HbA2 : 2 alpha and 2 epsilon chains

1.5–3% of adult haemoglobin

HbF : 2 alpha and 2 delta chains

1% of adult haemoglobin

70–90% at term, fall to 25% by 1st month and progressively

First discovery in 1954

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Alpha ThalassemiaGenes delation on chromosome 16

2

Normally, there are 4 genes for alpha globin with 2 genes on each chromosome

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Alpha ThalassemiaGenes delation on chromosome 16

Silent carrier [3 functional alpha globin genes]

asymptomatic

Alpha Thalassemia Trait [2 functional alpha globin genes]

no anemia / mild anemia

microcytosis

Hemoglobin H disease [1 functional alpha globin gene]

microcytosis & hemolysis (results in severe anemia)

bone deformities (overcompenasation by bone marrow)

splenomegaly (peripheral hemolysis)

Golf ball inclusions on microscopy

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Alpha ThalassemiaGenes delation on chromosome 16

Alpha Thalassemia Major [no functional alpha globin genes]

HB Bart’s [high oxygen affinity]

fatal hydrops fetalis [edema and ascites due to accumulation

serous fluid as result of severe anemia]

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Beta ThalassemiaGenes mutation on chromosome 11

Hundreds of mutations possible in the beta thalassemia, therefore

beta thalassemia is more diverse

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Beta ThalassemiaGenes mutation on chromosome 16

Silent carrier

Beta Thalassemia Minor (Trait)

asymptomatic

microcytosis, minor anemia [Hb >10 g/dL]

Beta Thalassemia Intermedia

less severe to Cooley anemia [Hb 8–10 g/dL]

Beta Thalassemia Major (Cooley anemia)

moderate to severe anemia [Hb <7mg/dL]

present younger than 2 years old

bone deformities (overcompenasation by bone marrow)

splenomegaly (peripheral hemolysis)

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Thalassemia

เกณการจย

1. ภาวะเอดจางชด microcytic anemia; MCV <80 fL

2. กษณะ hypochromic, anisocytosis, poikilocytosis

3. อาการทางคก: thalassemia facies, hepatosplenomegaly

4. อาจระบ serum ferritin ปกหอง

5. อาจผลตรวจ hemoglobin electrophoresis

ไองนกและใรสจย

: D64.9 Anemia, unspecified

: D63.8 Anemia in other chronic disease classified elsewhere

‘Thalassa' = Great Sea (Mediterranean sea)

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Thalassemia

Summary Diagnosis ICD-10

Alpha thalassemia [Alpha thalassemia major]

Hemoglobin H disease [Alpha thalassemia intermedia]

Hemoglobin constant spring

D56.0

Beta thassemia [Beta thalassemia major]

Beta thalassemia / Hemoglobin E

Beta thalassemia intermedia

D56.1

Alpha thalassemia trait [Alpha thalassemia minor]

Beta thalassemia trait [Beta thalassemia minor]

Thalassemia trait [Thalassemia minor]

D56.3

Homozygous hemoglobin E

Hemoglobon E traitD58.2

Thalassemia D56.9

‘Thalassa' = Great Sea (Mediterranean sea)

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Thalassemia Complications

Transfusion-Transmitted Infections

Transfusion Iron Overload

Toxicities of Iron Chelation Therapy

Bacterial Infections

Cardiac Complications

Liver Complications

Bone Complications

Growth Retardation

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Iron Overload

Each unit of blood has 200 mg of iron

Iron loading rate depends upon number of units transfused

!

!

!

!

!

Hemosiderosis

: accumulation of hemosiderin, no significant tissue destruction

Hemochromatosis

: directly deposited in the tissue, may be genetic or acquired

GroupDaily Intake

mg/kg/d

Annual Intake of PRC

ml/kg

Low <0.3 <150

Moderate 0.3 - 0.5 150 - 250

High >0.5 >250

Hemosiderosis, Hemochromatosis, Iron poisoning

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Iron Overload

Iron poisoning

: acute overdose, may cause severe or even fatal symptoms due

to toxic effect of free iron in plasma which may be life threatening

: nausea, vomiting, abdominal pain, diarrhea

Hemosiderosis, Hemochromatosis, Iron poisoning

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Acquired Hemochromatosis

Caused by excess intake of iron, increased hemolysis and

repeated blood transfusion

Related to the involved organ system

Secondary hemochromatosis

Liver: Cirrhosis

Pancreas: Diabetic mellitus

Skin: Pigmentation, Bronzed diabetes

Endocrine: Hypothyroidism, Testicular atrophy

Joints: Arthritis

Heart: Arrhythmia, Heart Failure

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Iron Overload

Summary Diagnosis ICD-10

Iron overload

Hemosiderosis

Hemochromatosis

Congenital hemochromatosis

Acquired hemochromatosis

Transfusion hemochromatosis

Transfusion hemosiderosis

Iron toxicities

R79.0

E83.1

E83.1

E83.1

E83.1

E83.1

T80.8

T45.4

Hepatic hemochromatosis

Diabetic hemochromatosis

Arthritis in hemochromatosis

Cardiac hemochromatosis

E83.1

E83.1

E83.1 + M14.5

E83.1

Hemosiderosis, Hemochromatosis, Iron poisoning

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Iron Chelation Therapy

Deferoxamine Deferiprone Deferasirox

Brand Name Deferal Ferriprox Exjade

Half-life 20 minutes 2-3 hours 8-16 hours

Route SQ, IV PO PO

Dose (mg/kg/d) 20-60 75-100 20-40

Frequency 5-7 days/week 3 times daily Once daily

Iron Excretion Urine/Stool Urine Stool

Side EffectsVision, Hearing, Growth,

Local Reactions, Allergy

GI Symptoms, Kidney

Dysfunction, Hepatitis

GI Symptoms,

Agranulocytosis/

Neutropenia. Arthralgia

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Autoimmune Hemolytic Anemia

Hemolytic Anemia

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Hymolytic Anemia

Normal RBC life 110–120 days

Anemia result from an increase in RBC destruction coupled with

increased erythropoiesis

Reduced RBC life span

The course of the disease Acute Chronic

The place of destruction Intravascular Extravascular

The whence Acquired Hereditary

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Predominantly Intravascular Hemolysis Predominantly Extravascular Hemolysis

Extracorpuscular (agents form outside the RBC)

Immune hemolysis: cold antibody

Microangiopathic hemolysis

Infectious agents, as in malaria

Thermal injury

Chemicals/Drugs

Venoms

Acute hemolytic transfusion

Prosthetic heart valves

Immune hemolysis: warm antibody

Membrane abnormalities

Spur cell anemia of severe liver disease

Paroxysmal nocturnal hemoglobinuria

Membrane defects [spherocytosis]

Intracorpuscular (abnormalities of the RBC interior)

Enzyme defects [G6PD deficiency]

Globin abnormalities [sickle cell

blue = acute hemolysis red = chronic hemolysis

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Hemolytic Anemia

Summary Diagnosis ICD-10

Autoimmune hemolytic anemia (AIH)

Warm AIH

Cold AIH

Drug-induced AIH

D59.1

D59.1

D59.1

D59.0

Hemolytic-uremic syndrome

HELLP syndrome

D59.3

O1.42

Microangiopathic hemolytic anemia

Mechanical hemolytic anemia

Transfusion hemolysis

D59.4

D59.4

T80.8

Extracorpuscular

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Hemolytic Anemia

Summary Diagnosis ICD-10

Paroxysmal nocturnal hemoglobinuria D59.5

Spherocytosis

Stomatocytosis

Elliptocytosis

Ovalocytosis

D58.0

D58.8

D58.1

D58.1

Membrane abnormalities

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Hymolytic AnemiaReduced RBC life span

Intravascular hemolysis Extravascular hemolysis

Microangiopathic hemolysis

Paroxysmal nocturnal hemoglobinuria

Infections

Toxins

Hypersplenism

Hemoglobinopathies

Enzymopathies

Membrane defects

Autoimmune hemolytic anemia

Polychromasia

Reticulocyte count: increased

Unconjugated bilirubin: increased

Plasma free hemoglobin: normal

Urine bilirubin: +

Urine hemosiderin: +

Urine hemoglobin: +

Polychromasia

Reticulocyte count: increased

Unconjugated bilirubin: increased

Plasma free hemoglobin: increased

Urine bilirubin: +

Urine hemosiderin: 0

Urine hemoglobin: 0

H=hereditary; A=acquired

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