1. Red Blood Cell Disorders
Stephen Medlin D.O. FACP

2. High Yield Board Topics
Anemia and Presentations for Multiple Myeloma
Types of Anemia
B12 deficiency
Anemia of Inflammation
IV iron

3. Myeloma-Properties of Plasma Cells
Proliferate
Secrete Immunoglobulins
“Make space”
Influence bone turnover
Secrete Inflammatory mediators

4. 4

5. SPEP
polyclonal
monoclonal

6. Myeloma Presentations
65 year old patient with normocytic anemia (hgb 10.7, MCV 85), may or may not have symptoms
What is the next laboratory test most likely to obtain a diagnosis
Low Albumin and Normal Total Protein
Creatinine of 1.35
albumin 2.2
total protein 7.2
calcium level of 10.7 (12.4)
Answer is Serum Protein Electrophoresis or Bone Marrow Biopsy

7. Myeloma Presentations-variations
65 year old patient with normocytic anemia (hgb 10.7, MCV 85), may or may not have symptoms
Slightly Low Albumin and elevated Total Protein
Creatinine of 1.35
albumin 3.2
total protein 9.2
calcium level of 11.0
Easy if there is an imaging study with a lytic lesion
What is the next laboratory test most likely to obtain a diagnosis
Answer is Serum Protein Electrophoresis or Bone Marrow Biopsy

8. Same Stem as Prior Slide but with Acute Pain Requiring Imaging
Ten Causes for Renal Failure in Myeloma
Related to Monoclonal protein (only one per patient)
Amyloid formation
“Myeloma Kidney”-Intratubular cast formation of immunoglobulin leading to cast nephropathy (40-60% of MM cases)
Nephrotic syndrome due to amyloid / glomerular damage
Cryoprecipitates / vasculitis
Other causes for Renal Failure
Hypercalcemia with nephrocalcinosis
ATN secondary to dehydration with or without hypercalcemia
iv ct contrast (predisposes to light chain precipitation/cast nephropathy or can cause renal tubular precipitation of light chains) 1.5% of the pt with myeloma develop this
uric acid nephropathy
plasma cell infiltration
NSAIDs

9. Anemia-Background Normal Erythropoiesis
Erythropoietin regulates rbc production
Immature rbc= reticulocytes
RBC’s pass through spleen and old or damaged cells are ingested by macrophages, iron transported to liver
Iron absorption is through the gut by the protein ferroportin which releases to transferrin
Hepcidin decreases absorption by binding ferroportin and increases with inflammation (IL-6 especially)

10. Red Blood Cell Disorders
Workup of Iron Deficiency
IDA
Thalassemia
Anemia of Inflammation
Diagnosing B12 deficiency
Unsteady gait/neurologic deficient
Macrocytic anemia/pancytopenia

11. Workup of Anemia MCV is the 1st step in anemia workup
Categorize into microcytic, normocytic, macrocytic group
Microcytic has a narrow differential
Macrocytic has a narrow differential
Hyperprolilferative (>2%) versus Hypo-proliferative is also a helpful distinction
IDA is HYPO-proliferative
Retic count more than 5% should trigger consideration of hemolysis

12. Approach to Microcytosis
Check Iron Studies
Serum Fe, TIBC, %sat
Ferritin (gold standard)
Watch for ESR elevation
(Anemia of inflammation)
Hemoglobin electrophoresis may be the answer if ferritin is normal and esr normal

13. Evaluation of Anemia
Frequently Fe Studies are not clear (% sat is low is NOT SPECIFIC)
Ferritin is KEY-NO FALSE LOW FERRITIN
65 year old female with fatigue, 85 kg
WBC Hemoglobin 9.0 L
MCV L Hematocrit 27.7 L
Platelets 319 Creatinine
Ferritin 160, iron % sat low (14) and TIBC normal or low (250), ESR 80
Normal Transaminases (elevated transaminases cause falsely elevated ferritin)
Anemia of Inflammation/Chronic Disease

14. Evaluation of Anemia 65 year old female with Answer: Iron Deficiency
WBC Hemoglobin 9.0 L
MCV L Hematocrit 27.7 L
Platelets 319
Ferritin 15, iron % sat low (14) and TIBC high (450)
ESR 20
Answer: Iron Deficiency
A simplistic stem may ask diagnosis but more likely is a management question “The patient has already been on oral iron and anemia has not improved, what is the next best step?”
Bone marrow
B12 administration
pursue workup-colonoscopy

15. Gestational Anemia
Typically results in Hgb more than 11 in the first and third trimesters and greater than 10.5 in the second trimester
In pregnant women without obvious blood loss, and iron deficiency, no further workup is warranted
Oral iron supplementation
Make sure the patient is on folic acid supplementation

16. Evaluation of Anemia 45 year old male with chronic anemia
WBC: 5.1, Hemoglobin: 13.7, Hematocrit: 40.7, MCV: 73.5 (L), Platelets: 197
Iron: 54, Ferritin: 285.8, % Iron Saturation: 17.3, TIBC: 312
ESR 13, normal transaminases
Question would be “what is the best test to obtain the most likely diagnosis”
Hemoglobin Electrophoresis
Sickle Thal Trait

17. Sickle Cell Questions on Key Points
IV fluids and adequate pain management are key points
Preoperative transfusion is recommended for major surgery with patients with Hb SS (GOAL is 10)
Low risk procedures such as skin biopsies, anesthesia with imaging not included
Pregnancy
SS anemia have increased complications but routine transfusions not helpful
Severely anemic and signs of heart failure are indications
Opiates are appropriate in pregnancy (except meperidine) but no hydroxyurea

18. Macrocytic Anemia Evaluate Macrocytic anemia B12/folate
Hemolytic workup-could be Retic’s
Ldh,haptoglobin, periph.smear, retic count, coombs test
Review drug list-Hydroxyurea classic
Mcv often over 110
Liver disease
Etoh hx
Splenomegaly ?
Myelodysplasia
Need BM biopsy to confirm

19. B12 Pearls
Elevated serum methylmalonic acid is more sensitive and specific than B12 levels
Oral B12 is an option
Folate can correct anemia in B12 deficiency but not neurologic symptoms