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Hematology Case
Conference
8 October 2015
Patient MS
56-year-old man with a history of JAK2 V617F+ ET,
presenting with severe anemia
Patient MS
56-year-old man with a history of JAK2 V617F+ ET,
presenting with severe anemia
• 2000: Diagnosed with ET, star...
Patient MS
• 8/26/2015: Presented to UCC with fevers, night
sweats, fatigue, weight loss
• WBC 2.9 (ANC 1.6, 1.7% blasts)
...
Patient MS
• 8/26/2015: Presented to UCC with fevers, night
sweats, fatigue, weight loss
• WBC 2.9 (ANC 1.6, 1.7% blasts)
...
Patient MS
• 8/28/2015: BMA/Bx
• Hypercellular marrow with left-shifted erythroid
hyperplasia accounting for ~ 80% cellula...
Patient MS
• Rest of the workup
• Total Fe 107, TIBC 185, 55% sat, UIBC 89
• Ferritin 505
• Reticulocytes 2.3%, absolute 5...
Patient MS
• 8/27/2015: DAT
• IgG weak+, C3 weak+
• Eluate negative
Patient MS
• 8/27/2015: DAT
• IgG weak+, C3 weak+
• Eluate negative
• 9/1/2015: Repeat DAT
• IgG weak+, C3 weak+
• Eluate ...
Patient MS
• 8/27/2015: DAT
• IgG weak+, C3 weak+
• Eluate negative
• 9/1/2015: Repeat DAT
• IgG weak+, C3 weak+
• Eluate ...
Patient MS
• 9/11/2015: Presented to UCC with fevers, dyspnea,
abdominal pain
• WBC 1.8 (ANC 0.9, no blasts)
• Hgb 6.4 (MC...
Patient MS
• 9/11/2015: In UCC
• Lactate 10.8
• AST 171 (ALT normal), K 5.3
• Cr 1.4 (baseline 1.0), eventually up to 5.9
...
Patient MS
• 9/11/2015: Repeat DAT
• IgG 2+, C3 4+
• Eluate +panagglutinin
• Anti-K identified; anti-E cannot be ruled out...
In Summary
Concurrent warm autoantibody-mediated hemolytic
anemia and delayed hemolytic transfusion reaction
in a patient ...
Red Cell Transfusion
Reactions
• Anaphylactic
• Acute hemolytic
• Delayed hemolytic
• Febrile nonhemolytic
Blood Group Systems
Lichtman et al., Williams Hematology, 8th ed.
Blood Group Systems
Lichtman et al., Williams Hematology, 8th ed.
Blood Group Systems
Lichtman et al., Williams Hematology, 8th ed.
Blood Group Systems
Lichtman et al., Williams Hematology, 8th ed.
The
Kell
Syste
m
Blood 1953
The Kell Blood Group
System
• Discovered by Coombs et al. in 1946
• Named after patient Kelleher, mother of an infant
unde...
The Kell Blood Group
System
Antigens
• K, k
• Kpa, Kpb
• Jsa, Jsb
• K11, K17
• K14, K24
Yellow = high incidence Ag
Westhof...
The Kell Blood Group
System
• K: Incidence 9% in Caucasians, 2% in African
Americans
• After A/B and RhD, K demonstrates h...
Anti-K
Alloimmunization
• Sanz et al., Transfusion 2013: Series of 272
transfusion-dependent patients with MDS or
CMML
• M...
Anti-K
Alloimmunization
• Sanz et al., Transfusion 2013: Series of 272
transfusion-dependent patients with MDS or
CMML
• M...
Anti-K in Fetal
Anemia
• With prophylaxis against RhD alloimmunization,
anti-Kell responsible for almost 10% of antibody-
...
NEJM 1998
Anti-K in Fetal
Anemia
• Proposed inhibition of
megakaryopoiesis by
anti-K
• Mean platelet
count in 3 fetuses
with anti-K
...
Anti-K in Fetal
Anemia
• Proposed expression of Kell antigens on myeloid
progenitor cells and anti-Kell inhibition of CFU-...
J Pediatr Hematol Oncol 2004
Anti-K in Fetal
Anemia
Back to the Patient
• Admitted to ICU
• Course complicated by NSTEMI, acute kidney injury
• Started on glucocorticoids for...
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Heme conf 10 08-2015 - dr cho

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Heme conf 10 08-2015 - dr cho

  1. 1. Hematology Case Conference 8 October 2015
  2. 2. Patient MS 56-year-old man with a history of JAK2 V617F+ ET, presenting with severe anemia
  3. 3. Patient MS 56-year-old man with a history of JAK2 V617F+ ET, presenting with severe anemia • 2000: Diagnosed with ET, started anagrelide • 2003: Switched to hydroxyurea • 2015: Hydroxyurea discontinued for progressive anemia • Since 6/2015: Hgb 10 → 8.7 → 6.9 (8/19) (remaining counts not available) • First pRBC transfusion 8/19/2015
  4. 4. Patient MS • 8/26/2015: Presented to UCC with fevers, night sweats, fatigue, weight loss • WBC 2.9 (ANC 1.6, 1.7% blasts) • Hgb 7.7 (MCV 94) • Plts 183,000
  5. 5. Patient MS • 8/26/2015: Presented to UCC with fevers, night sweats, fatigue, weight loss • WBC 2.9 (ANC 1.6, 1.7% blasts) • Hgb 7.7 (MCV 94) • Plts 183,000 • Hospitalized from 8/26 to 9/5 • Transfusion-dependent anemia, received 8 units pRBCs total
  6. 6. Patient MS • 8/28/2015: BMA/Bx • Hypercellular marrow with left-shifted erythroid hyperplasia accounting for ~ 80% cellularity • Trilineage dysplasia • 2+ fibrosis by reticulin stain • 1% blasts • FISH: Negative for del(13q), del(20q) • DMP: JAK2 V617F+, p53 exon 5 fs mutation • Consistent with evolution to MDS, close follow- up recommended to exclude progression to high-grade myeloid disorder such as AEL
  7. 7. Patient MS • Rest of the workup • Total Fe 107, TIBC 185, 55% sat, UIBC 89 • Ferritin 505 • Reticulocytes 2.3%, absolute 57.9 • Haptoglobin < 1 • LDH 1,300s-1,500s • T.bili to 1.9 (70% unconjugated)
  8. 8. Patient MS • 8/27/2015: DAT • IgG weak+, C3 weak+ • Eluate negative
  9. 9. Patient MS • 8/27/2015: DAT • IgG weak+, C3 weak+ • Eluate negative • 9/1/2015: Repeat DAT • IgG weak+, C3 weak+ • Eluate +panagglutinin
  10. 10. Patient MS • 8/27/2015: DAT • IgG weak+, C3 weak+ • Eluate negative • 9/1/2015: Repeat DAT • IgG weak+, C3 weak+ • Eluate +panagglutinin • Continued transfusions, discharged home 9/5 • Outpatient CBCs post-discharge: Hgb 7.9-8.5
  11. 11. Patient MS • 9/11/2015: Presented to UCC with fevers, dyspnea, abdominal pain • WBC 1.8 (ANC 0.9, no blasts) • Hgb 6.4 (MCV 94) • Plts 192,000 • Smear: Rouleaux, no schistocytes • Reticulocytes 4.5%, absolute 87.3 • Haptoglobin < 1 • LDH 5,040 • T.bili 2.3
  12. 12. Patient MS • 9/11/2015: In UCC • Lactate 10.8 • AST 171 (ALT normal), K 5.3 • Cr 1.4 (baseline 1.0), eventually up to 5.9 • CTAP without bleed or other acute process • Started on pip/tazo and transfused • 1st unit pRBCs → Hgb 5.0 • 2nd unit pRBCs → Hgb 5.6
  13. 13. Patient MS • 9/11/2015: Repeat DAT • IgG 2+, C3 4+ • Eluate +panagglutinin • Anti-K identified; anti-E cannot be ruled out • Patient had received 2 units of K+ pRBCs (9/2, 9/3) and 1 unit RhE+ (9/5) during his previous admission
  14. 14. In Summary Concurrent warm autoantibody-mediated hemolytic anemia and delayed hemolytic transfusion reaction in a patient with MF/MDS
  15. 15. Red Cell Transfusion Reactions • Anaphylactic • Acute hemolytic • Delayed hemolytic • Febrile nonhemolytic
  16. 16. Blood Group Systems Lichtman et al., Williams Hematology, 8th ed.
  17. 17. Blood Group Systems Lichtman et al., Williams Hematology, 8th ed.
  18. 18. Blood Group Systems Lichtman et al., Williams Hematology, 8th ed.
  19. 19. Blood Group Systems Lichtman et al., Williams Hematology, 8th ed.
  20. 20. The Kell Syste m Blood 1953
  21. 21. The Kell Blood Group System • Discovered by Coombs et al. in 1946 • Named after patient Kelleher, mother of an infant under evaluation for HDN • 30 known antigens • All antigens carried on Kell glycoprotein • 3,500-17,000 copies per RBC • Encoded on chromosome 7q33 • Expressed early during erythropoiesis • Also found in brain, testis, and lymphoid tissues • Member of neprilysin family of Zn endopeptidases
  22. 22. The Kell Blood Group System Antigens • K, k • Kpa, Kpb • Jsa, Jsb • K11, K17 • K14, K24 Yellow = high incidence Ag Westhoff and Reid, Immunohematology 2004
  23. 23. The Kell Blood Group System • K: Incidence 9% in Caucasians, 2% in African Americans • After A/B and RhD, K demonstrates highest immunogenicity among the RBC antigens • K stimulates anti-K in 10% of cases • Other antibodies less common • Anti-Ku is “total Kell” antigen, produced by individuals who are K0
  24. 24. Anti-K Alloimmunization • Sanz et al., Transfusion 2013: Series of 272 transfusion-dependent patients with MDS or CMML • Median 33 units of pRBCs • 15% of patients formed alloantibodies (n = 42) • Cumulative incidence 19.5% at >= 130 units • Anti-K most common (26 cases), followed by E (19), c (5), and Jka (5)
  25. 25. Anti-K Alloimmunization • Sanz et al., Transfusion 2013: Series of 272 transfusion-dependent patients with MDS or CMML • Median 33 units of pRBCs • 15% of patients formed alloantibodies (n = 42) • Cumulative incidence 19.5% at >= 130 units • Anti-K most common (26 cases), followed by E (19), c (5), and Jka (5) * Detection can be masked if panagglutinin present
  26. 26. Anti-K in Fetal Anemia • With prophylaxis against RhD alloimmunization, anti-Kell responsible for almost 10% of antibody- mediated severe fetal anemia • Fetal anemia in the setting of anti-K is not mediated solely by hemolysis • Anti-K titers and bili are poor predictors of anemia • Circulating reticulocyte/normoblast and amniotic fluid bili < those in anti-RhD alloiummunized infants
  27. 27. NEJM 1998
  28. 28. Anti-K in Fetal Anemia • Proposed inhibition of megakaryopoiesis by anti-K • Mean platelet count in 3 fetuses with anti-K erythroblastosis fetalis 63 ± 5 x 109 • vs. 252 ± 15 x 109 in 5 fetuses with anti-D Wagner et al., NEJM 2000
  29. 29. Anti-K in Fetal Anemia • Proposed expression of Kell antigens on myeloid progenitor cells and anti-Kell inhibition of CFU-GM growth Wagner et al., BJH 2000
  30. 30. J Pediatr Hematol Oncol 2004 Anti-K in Fetal Anemia
  31. 31. Back to the Patient • Admitted to ICU • Course complicated by NSTEMI, acute kidney injury • Started on glucocorticoids for warm AIHA • Supportive care for DHTR • Hgb improved within days • Discharged home 9/22 • Readmitted 10/3 with fever; anemia persistent but stable; thrombocytopenia to 10s

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