Intended for Healthcare professionals only. Initiated and funded by Alexion

Common symptoms
for something uncommon

When you see patient with signs and symptoms such as:

Fatigue
Abdominal pain
Dyspnoea
Erectile dysfunction
Dysphagia
Anaemia

What do you consider as a possible cause?

See below

Did you know that:

44% of patients

with Paroxysmal Nocturnal
Haemoglobinuria (PNH) have
abdominal pain at presentation¹

80% of patients

with PNH present with fatigue¹

What is Paroxysmal Nocturnal Haemoglobinuria (PNH)?

Paroxysmal Nocturnal Haemoglobinuria (PNH) is a chronic, devastating disease.
In PNH, dysregulated terminal complement activation leads to intravascular haemolysis (IVH) and thrombophilia, causing premature mortality and significant morbidity.^{2, 3, 4}

PNH symptoms are non-specific,
and coupled with the rarity of PNH, can lead to substantial diagnostic delays.^{5, 6, 7}

Watch to learn more about PNH

Typical signs and symptoms of PNH

The signs and symptoms of PNH are very variable and may appear with decreasing frequency in the form of fatigue, anaemia, dyspnoea, haemoglobinuria, abdominal pain, erectile dysfunction, and dysphagia.

Image showing typical signs and symptons of PNH

40%–67%

of PNH deaths are due to venous or arterial thrombosis^{11, 12}

8-18%

of PNH deaths
are caused by
renal failure¹¹

47%

suffer from
pulmonary
hypertension¹⁰

However, a quick intervention could save your patients.^{13, 14}

Why early diagnosis
of PNH is needed

Rapidly recognising the signs and symptoms of PNH and achieving early diagnosis are vital for disease management.^{13, 14}

Without complement-targeting therapy, PNH has a poor prognosis.¹⁵

As a result, 20–35% of untreated patients with PNH die within 5–6 years of diagnosis.^†11

PNH patient case studies coming soon

Testing/Referral

Lactate dehydrogenase (LDH) is the established biomarker of intravascular haemolysis in PNH.¹⁶

If LDH is ≥1.5 x upper limit of normal (ULN), PNH can be suspected.¹⁷

Patients with LDH ≥1.5 x (ULN) are:

7x

more likely to develop thromboembolic events than patients with LDH <1.5 x ULN.¹⁷

5-fold

Have a 5-fold higher mortality rate compared with the general population.¹³

In the presence of signs and symptoms of PNH, consider ordering additional tests, and refer to a haematologist and follow up to manage underlying internal conditions.^{7, 18}

You play a major role in the pathway to diagnosis⁷

Evaluate

Test

Refer

LDH: Lactate dehydrogenase
*Please note that there are regional variances on which healthcare professionals can order testing.
The information on this page is intended as educational information for healthcare providers. It does not replace a healthcare professional’s judgement or clinical diagnosis.

Patient presents

GP evaluates the patient

GP identifies signs and symptoms relates to PNH
(fatigue, anaemia, dysphagia, dyspnoea, back pain, erectile dysfunction, haemoglobimuria, haematuria, pulmonary hypertension)

Do you have any questions about PNH?

References:

Scherezenmeier H, et al. Haematologica. 2014;99(5):922-929 and Supplemental Information.
Risitano AM, et al. Blood. 2009;113(17):4094 - 4100.
Berentsen S, et al. Ther Adv Hematol. 2019;10:1–20.
Hill A, et al. Blood. 2013;121(25):4985 - 4996.
Parker CJ. Am Soc Hematol Educ Program. 2011;2011:21–29.
Hillmen P, et al. N Engl J Med. 1995;333(19):1253-1258.
Sahin F, et al. Am J Blood Res. 2015;5(1):1-9.
Nishimura J, et al. Medicine (Baltimore). 2004;83(3):193-207.
Mitchell R, et al. SM Clin Med Oncol. 2017;1(1):1001.
Hill A, et al. Br J Haematol. 2010;149(3):414-425.
Hillmen P, Muus P, Röth A, et al. Br J Haematol. 2013;162(1):62–73.
Sharma VR. Clin Adv Hematol Oncol. 2013;11 Suppl 13(9):2-8.
Jang JH, Kim JS, Yoon SS, et al. J Korean Med Sci. 2016;31:214–221.
Borowitz MJ, Craig FE, DiGiuseppe JA, et al. for Clinical Cytometry Society. Cytometry B Clin Cytom. 2010;78B:211–230.
Loschi M, Porcher R, Barraco F, et al. Am J Hematol. 2016;91(4):366–370.
Risitano AM, et al. Front Immunol. 2019;10:1157.
Lee JW, et al. Int J Hematol. 2013;97(6):749–757.
Barcellini W, Fattizzo B. Dis Markers. 2015;2015:635-670.

Job code: CH/UNB-P/0002
Date of preparation: June 2021

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Common symptoms for something uncommon