ARBIE C

Arbie C, age 43, is a popular DJ from Nigeria who immigrated

to Australia 14 months ago. He does not smoke or drink
alcohol, and has no allergies. He has a past medical history of
vesicoureteric reflux since childhood, for which he has taken
prophylactic amoxicillin for many years. He takes no other
regular medications.
Since coming to Australia, he has been hospitalised three times
with urinary tract infections, the last two of which were
complicated with sudden and severe anaemia. He recovered
each time over about two weeks, but given the recurrence of
the anaemia, his discharge summary from the last admission (4
days ago) asks his GP to follow up his iron studies and FBC
and to consider further workup, colonoscopy, and possibly a
transfusion if Arbie is still symptomatic. His results are
attached.

Is Arbie anaemic?

Is Arbie iron deficient?

Should Arbie have an iron infusion?

Should Arbie have a blood transfusion?

Should Arbie have a colonoscopy?

What is your differential diagnosis list to explain Arbie’s

blood loss? Give at least three likely diagnoses that these
results could support.

What other tests could help rule in or rule out your DDx?

How will you counsel Arbie?

ARBIE C
Arbie C, age 43, is a popular DJ from Nigeria who immigrated
to Australia 14 months ago. He does not smoke or drink
alcohol, and has no allergies. He has a past medical history of
vesicoureteric reflux since childhood, for which he has taken
prophylactic amoxicillin for many years. He takes no other
regular medications. He is fully immunised.
Since coming to Australia, he has been hospitalised three times
with urinary tract infections, the last two of which were
complicated with sudden and severe anaemia. He recovered
the first time over about two weeks, but given the recurrence of
the anaemia, his discharge summary from this last admission
(4 days ago) asks his GP to follow up his iron studies and FBC
and to consider further workup, colonoscopy, and possibly a
transfusion if Arbie is still symptomatic. His results are
attached.

Arbie is feeling much better but still is easily fatigued and mildly
breathless on exertion. Examination today is unremarkable
and he is vitally stable.
Is Arbie anaemic?

Yes, his haemoglobin dropped precipitously both times. He still

has not yet recovered to normal levels of haemoglobin.

His results show a normocytic, normochromic (actually

hyperchromic) anaemia.

What are the causes of normocytic anaemia?

Blood loss, haemolysis, or marrow problems with production

(for example leukaemia, lymphoma, myeloma, myelofibrosis,
myelodysplasia)
Is Arbie iron deficient?

No, Arbie is absorbing sufficient iron (serum iron) and storage

iron (ferritin.) His iron transport is not accelerated (transferrin).

Should Arbie have an iron infusion?

No, since he is not deficient, it will not help his anaemia, and it
may harm him by contributing to iron overload.

What is your differential diagnosis list to explain Arbie’s

blood loss? Give at least three likely diagnoses:

Normocytic normochromic anaemia can be seen in chronic

disease or rapid blood loss. The timing does not support
chronic disease, and the near-normal ferritin also goes against
this.

RBCs can be lost by bleeding or by haemolysis. Occult

bleeding is a possibility, but does not fit the timing here. Rapid
blood loss of that amount should be detectable.

This leaves us with haemolysis of some sort as the explanation:

drug-induced haemolytic anaemia AIHA, malignancy, toxicity,
G6PD deficiency, porphyria, spherocytosis, sickle cell anaemia.

Which results contribute the most to forming your

diagnostic short list?

There are only a few things which raise the MCHC in

anaemia MCHC in the presence of anaemia. Many unrelated
causes of a high MCHC have haemolysis as a common
mechanism. (The total haemoglobin stays relatively constant,
but the cell numbers go down, meaning the Hb per cell goes
up.)
What other tests could help rule in or rule out your DDx?

Tests to confirm haemolysis: LDH, haptoglobin, bilirubin

Tests to assess the cause of haemolysis: autoimmune versus

cellular issues

AIHA: Coombs test/ DAT, complement tests, ANA/ENA

Membrane issues: film exam for spherocytes, sickle cells,

fragments, Hb electrophoresis

Cellular damage: G6PD level, reticulocyte count

Malignancy: film exam for blasts and other abnormal cells

Should Arbie have a blood transfusion?

If AIHA, transfusion is contraindicated. In other forms of

haemolytic anaemia, transfusion can sometimes be helpful.

It would be safe for Arbie to have a transfusion, but it is not

necessary as he is improving clinically and he has sufficient
iron stores to “restock the shelves.”

Should Arbie have a colonoscopy?

No, his anaemia is explained and he is not at increased risk for

GI blood loss. He should be screened for colorectal cancer at
age 50 with FOBT.

FURTHER CASE INFO:

Arbie had further testing with you and was shown to have
G6PD deficiency, making him susceptible to haemolytic
episodes in response to oxidative stresses, most often drugs:
he was given ceftriaxone for the first UTI, which did not trigger
a crisis, however he was given nitrofurantoin for the most
recent two infections, and this drug is notorious for triggering
haemolytic episodes in people with G6PD.
Assuming the trigger goes away, these haemolytic episodes do
self-resolve as the marrow produces healthy young
reticulocytes that rapidly offset the anaemia as well.

How will you counsel Arbie?

We need to get to the bottom of the recurrent UTIs!

His sexual habits have changed now that he is in Australia

(multiple partners, anal sex with men and women. You
counsel him on the importance of using condoms (He always
does with men but not with women as he perceives it to be
“safe” from a pregnancy POV and he perceives no HIV risk
from women in Australia. In Nigeria he uses condoms every
time because he perceives more of an HIV risk.)

We may need to change his prophylaxis medication

You can try cephalexin or triprim in G6PD but not Bactrim.

He needs to avoid oxidative stressors

a short list of medications as well as infections.

His daughters’ sons are at risk of inheriting this X-linked

disease.

All his daughters will be carriers

All his sons inherit his Y chromosome, not his affected X
AT DISCHARGE – four days ago
Group Detail Date Value w/Units Flags Normal Range
Chemistries Sodium 24/09/2019 136 mmol/L 135-145
Potassium 4.0 mmol/L 3.5-5.2
Chloride 111 mmol/L HI 95-110
Bicarbonate 17 mmol/L LOW 22-32
Anion Gap 12 mmol/L 7-17
Urea Level 4.8 mmol/L 4.0-9.0
Creatinine umol/L 70 umol/L 45-90
Protein Total 65 g/L 60-80
Albumin Level 36 g/L 30-44
Globulin 25 g/L 22-42
Bilirubin Total 36 umol/L HI <20
GGT 12 unit/L 5-35
ALP 47 unit/L 30-110
ALT 22 unit/L 10-35
AST 80 unit/L HI 10-35
Lactate 204 unit/L 120-250
Dehydrogenase
C Reactive Protein 28 mg/L HI <5

Iron Studies Iron 24 umol/L 8-30

Transferrin 1.8 g/L 1.8-3.5
Ferritin 316 ug/L HI 30-300
Transferrin 47 % HI 15-45
Saturation
Folate Level 10.3 nmol/L 9.3-52.6
Vitamin B12 Level 751 pmol/L HI 138-652
Haptoglobin 0.15 g/L LOW 0.30-2.00

Haematology Hb 69 g/L LOW 115-165

RCC 2.04 x10^12/L LOW 3.80-5.80
HCT 0.191 L/L LOW 0.320-0.460
MCV 93 fL 80-100
MCH 34 pg HI 27-32
MCHC 373 g/L HI 310-360
RDW 18.6 % <15

WCC Corrected 10.2 10^9/L 4.0-11.0

Neutrophils Absolute 8.8 x10^9/L HI 2.0-8.0
Lymphocytes 0.8 x10^9/L LOW 1.0-4.0
Absolute
Monocytes Absolute 0.4 x10^9/L 0.2-1.0
Eosinophils Absolute 0.2 x10^9/L <1.0
Basophils Absolute 0.0 x10^9/L <1.0
1.0
PLT 167 x10^9/L 150-400
MPV 6.7 fL LOW 7.2-11.1

Transfusion Blood Group Nil detected.

Medicine and Antibody Screen
Direct Antiglobulin Negative
Blood Group O POSITIVE
ON ADMISSION – nine days ago
Group Detail Date Value w/Units Flags Normal
Range
Chemistries Sodium 19/09/2019 09:55:03 135mmol/L 135-145
Potassium 4.1 mmol/L 3.5-5.2
Chloride 105 mmol/L 95-110
Bicarbonate 18 mmol/L LOW 22-32
Anion Gap 14 mmol/L 7-17
Urea Level 23.0 mmol/L HI 4.0-9.0
Creatinine umol/L 99 umol/L HI 45-90
Protein Total 64 g/L 60-80
Albumin Level 35 g/L 30-44
Globulin 219 g/L 22-42
Bilirubin Total 6 umol/L <20
GGT 12 unit/L 5-35
ALP 47 unit/L 30-110
ALT 22 unit/L 10-35
AST 80 unit/L HI 10-35
Lactate Dehydrogenase 514 unit/L HI 120-250
C Reactive Protein 156 mg/L HI <5
Haptoglobin 0.65 g/L 0.30-2.00

Haematology Hb 70 g/L LOW 115-165

RCC 2.02 x10^12/L LOW 3.80-5.80
HCT 0.191 L/L LOW 0.320-
0.460
MCV 94 fL 80-100
MCH 34 pg HI 27-32
MCHC 365 g/L HI 310-360
RDW 13.7 % <15

WCC Corrected 10.4 10^9/L 4.0-11.0

Neutrophils Absolute 9.1 x10^9/L HI 2.0-8.0
Lymphocytes Absolute 0.6 x10^9/L LOW 1.0-4.0
Monocytes Absolute 0.4 x10^9/L 0.2-1.0
Eosinophils Absolute 0.2 x10^9/L <1.0
Basophils Absolute 0.0 x10^9/L <1.0
1.0
PLT 155 x10^9/L 150-400
MPV 7.2 fL LOW 7.2-11.1

AT PREVIOUS DISCHARGE three months ago

Haematology Hb 14/06/2019 10:15:00 108 g/L LOW 115-165
RCC 2.56 x10^12/L LOW 3.80-5.80
HCT 0.238 L/L LOW 0.320-
0.460
MCV 93 fL 80-100
MCH 34 pg HI 27-32
MCHC 369 g/L HI 310-360
RDW 13.6 % <15
GRAPHS OF TRENDS:

The red cell count dips along with the haemoglobin but more strongly, since haemolysis is happening
there are fewer cells to count.

The average RBC size stays fairly constant – there are so many RBCs it take a lot to shift this average.

The haemoglobin concentration goes abnormally high with each episode of haemolysis.
The RBC width distribution goes higher when haemolysis creates tiny cell fragments as well as the
normal sized RBCs – too much variety to be normal. (Also, reticulocytes are big and fat and add to
the variability of size.)