The following blood results are from a 51 -year-old female who presented with arthralgia, jaundice and dark urine.
Parameter |
Patient Value |
Normal Adult Ran e |
|||
Haemoglobin |
68 g/L |
115 - 160 |
|||
White Cell Count |
33.1 x 109/L |
4.0 — 11.0 |
|||
Platelets |
307 x 109/L |
150 - 400 |
|||
Red Cell Count |
2.22 x 1012/L * |
3.80 - 4.80 |
|||
Haematocrit |
0.19* |
0.37 - 0.47 |
|||
Mean Cell Volume |
88 fL |
80 - 100 |
|||
Mean Cell Haemoglobin |
30.7 |
27.0 - 32.0 |
|||
Mean Cell Haemoglobin Concentration |
350 g/L |
320 - 360 |
|||
Reticulocyes |
2.5%* |
0.2 - 2.0 |
|||
Reticulocytes Absolute |
56 x 109/L |
20 - 130 |
|||
Comment: The red cells show moderate numbers of spherocytes |
|||||
Serum Haptoglobins |
< 0.10 g/L |
0.30 - 2.00 |
|||
Serum Lactate Dehydrogenase |
948 U/L* |
140 - 280 |
|||
Total Protein |
69 g/L |
60 - 80 |
|||
Albumin |
33 g/L |
35 - 50 |
Globulins |
36 g/L |
25 - 42 |
|
Total Bilirubin |
119 mol/L* |
< 20 |
|
Alanine Aminotransferase |
276 U/L* |
< 35 |
|
Alkaline Phosphatase |
120 U/L* |
30 - 1 10 |
|
Gamma-GlutamyI Transferase |
114 U/L* |
< 40 |
|
Direct Antiglobulin Test: |
|||
IgG |
Positive |
||
C3d |
Positive |
a) What is the diagnosis? (20% marks)
b) List three treatment options. (15% marks)
a)
b)
a) The only possible answer is autoimmune haemolytic anaemia. Overall, the differential diagnosis of autoimmune haemolytic anaemias looks like this:
Warm haemolytic anaemia | Cold haemolytic anaemia |
|
|
In this scenario, the patient probably has one of the warm haemolytic anaemias, because both the IgG and C3d were found coating the red cells (IgM and C3d are found in cold haemolysis). The WCC is raised suggestive of a lymphoproliferative disorder as the cause. Arthralia may be due to some sort of autoimmune disease (SLE or RA), or it may be a subtle hint that the patient has been taking diclofenac to soothe her aching joints (which is a drug known to cause warm autoimmune haemolysis).
b)
Generic management of warm autoimmune haemolytic anaemia could have included any three of the following options:
Zeerleder, S. "Autoimmune haemolytic anaemia-a practical guide to cope with a diagnostic and therapeutic challenge." Neth J Med 69.4 (2011): 177-84.
Gehrs, Bradley C., and Richard C. Friedberg. "Autoimmune hemolytic anemia."American journal of hematology 69.4 (2002): 258-271.
Birgens, Henrik, et al. "A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia." British journal of haematology 163.3 (2013): 393-399.
Lechner, Klaus, and Ulrich Jäger. "How I treat autoimmune hemolytic anemias in adults." Blood 116.11 (2010): 1831-1838.
Salama, A., and B. Mayer. "Diagnostic pitfalls of drug-induced immune hemolytic anemia." Immunohematology 30.2 (2014): 80-4.
Zantek, Nicole D., et al. "The direct antiglobulin test: a critical step in the evaluation of hemolysis." American journal of hematology 87.7 (2012): 707-709.
Ma, Kim, and Stephen Caplan. "Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy." Case reports in hematology 2016 (2016).
Pignon, Jean‐Michel, Emmanuelle Poirson, and Henri Rochant. "Danazol in autoimmune haemolytic anaemia." British journal of haematology 83.2 (1993): 343-345.