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Eur J Case Rep Intern Med
European Journal of Case Reports in Internal Medicine
2284-2594
SMC Media Srl

10.12890/2024_004763
4763
Article
Severe Coagulopathy and Intra-Alveolar Haemorrhage Due to Fat Malabsorption in Celiac Disease
Lencovsky Ori 12
Katz-Talmor Daphna 1
Aronoff Benjamin 23
1 Internal Medicine Ward A, Samson Assuta Ashdod University Hospital, Ashdod, Israel
2 Nephrology and Hypertension Department, Samson Assuta Ashdod University Hospital, Ashdod, Israel
3 Department of Internal Medicine, Hackensack University Medical Center, Hackensack Meridian School of Medicine, Nutley, USA
Corresponding author’s e-mail: oril@assuta.co.il
2024
02 9 2024
11 9 00476311 7 2024
12 8 2024
© EFIM 2024
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This article is licensed under a Commons Attribution Non-Commercial 4.0 License
Celiac disease, a prevalent autoimmune disorder, can present atypically with fat malabsorption and coagulopathy due to vitamin K malabsorption.

A 64-year-old male presented with haemoptysis and severe anaemia (Hb 6 g/dl). Despite normal previous coagulation tests, admission laboratory tests revealed an international normalised ratio (INR) of 7.0 and iron deficiency anaemia. Initial blood products and vitamin K treatment corrected the INR temporarily, but the patient’s haemoptysis returned, and his INR values continued to rise. Further investigation revealed celiac disease with fat malabsorption, leading to vitamin K malabsorption and along with a previously prescribed antiplatelet aggregation therapy, this led to diffuse alveolar haemorrhage. A gluten-free diet and vitamin supplementation normalised the patient’s INR and stopped the bleeding.

This case highlights the importance of considering celiac disease in unexplained coagulopathies and the effectiveness of dietary management.

LEARNING POINTS

Celiac disease can cause severe coagulopathy due to fat malabsorption and vitamin K deficiency.

High suspicion is required for atypical presentations of celiac disease.

A gluten-free diet is essential for managing celiac disease and normalising coagulation profiles.

Celiac disease
coagulopathy
fat malabsorption
vitamin K deficiency
intra-alveolar haemorrhage
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pmcINTRODUCTION

Celiac disease is an autoimmune disorder affecting approximately 1.4% of the population[1]. It results from an immune response to ingested gluten, a protein found in wheat, barley and rye. This immune reaction leads to inflammation and damage to the small intestine’s mucosa, specifically the villi, which are responsible for absorption of nutrients. Celiac disease typically presents with a range of gastrointestinal symptoms, including chronic diarrhoea, abdominal pain, bloating and weight loss. Extraintestinal manifestations may include anaemia, osteoporosis, dermatitis herpetiformis and neurological symptoms such as peripheral neuropathy[2].

Damage to the villi impairs the absorption of fats and fat-soluble vitamins (A, D, E and K)[3]. Vitamin K is particularly crucial for the synthesis of clotting factors II, VII, IX and X. Without adequate vitamin K, the liver cannot produce functional clotting factors, reflected by prolonged prothrombin time and elevated international normalised ratio (INR), leading to coagulopathy and an increased risk of bleeding. Fat malabsorption also leads to deficiencies in vitamins A, D and E, contributing to a wide range of clinical symptoms including osteoporosis, night blindness and neuropathy[4,5].

CASE DESCRIPTION

A 64-year-old male presented at our emergency department with shortness of breath and haemoptysis, which started a day before his admission. He had been on dual antiplatelet therapy with prasugrel and aspirin for a recent acute coronary syndrome six months before admission, leading to percutaneous coronary intervention of his left circumflex artery and an insertion of a drug-eluting stent. He reported non-stop haemoptysis and shortness of breath, which worsened when lying down, and ruled out any recent trauma, change of medications, fever or melaena. He presented a plastic bag with about 100 ml of blood which he brought from home.

On physical examination, he was communicating and pale, with a respiratory rate of 15 breaths per minute, heart rate of 110 beats per minute, blood pressure of 135/70 mmHg and oxygen saturation of 83% on room air. Lung auscultation was significant for diffuse bilateral crackles; no other significant physical examination findings were noted. Initial laboratory tests on admission revealed severe anaemia (Hb: 6.0 g/dl), a significantly prolonged INR of 7.0. and prolonged activated partial thromboplastin time (aPTT). Further tests showed a low reticulocyte production index (0.34), normal platelet count and a normal D-dimer. Chemistry tests were significant for hypocalcaemia, slight hypomagnesaemia and hyponatraemia (Table 1). Iron studies were significant for iron deficiency as indicated by the very low transferrin saturation (7.9%) with normal ferritin levels (228 μg/l), and normal B12 and folic acid levels (Table 2).

A chest computerized tomography (CT) scan with IV contrast demonstrated bilateral emphysema (which was a known finding in previous CT’s of this patient) and diffuse alveolar haemorrhage (Fig. 1).

The patient was admitted to the intensive care unit and treated with vitamin K supplementation, blood transfusions and prothrombin complex concentrate, which temporarily normalised his INR. After the cessation of his haemoptysis, his respiratory status and haemodynamics stabilised, and he was transferred to the internal medical ward. Unfortunately, haemoptysis returned a week later with INR prolongation (Table 3), prompting further investigation. Further investigation was negative for antiphospholipid syndrome, ANCA-associated vasculitides and anti-GBM disease. A thromboelastogram showed results consistent with a coagulation factor defect; blood tests revealed hypocalcaemia and low levels of vitamin A, vitamin E and vitamin D, indicating potential fat malabsorption. The faecal elastase test was negative indicating no pancreatic insufficiency, and an abdominal CT imaging scan showed a normal pancreas and biliary tract. Elevated levels of anti-tissue transglutaminase antibodies were detected (Table 4). A duodenal biopsy revealed villous atrophy, increased intraepithelial lymphocytes and crypt hyperplasia, confirming the diagnosis of celiac disease. The combination of fat malabsorption markers, serology, iron deficiency anaemia and the biopsy findings confirmed celiac disease as the underlying cause. The patient was placed on a strict gluten-free diet. Parenteral vitamin K was continued until the coagulopathy was corrected. Over the following days his laboratory tests – including coagulation profiles – normalised, and he experienced no further bleeding episodes.

DISCUSSION

This case demonstrates an atypical presentation of celiac disease with severe coagulopathy due to impaired fat malabsorption and more specifically vitamin K deficiency. The severe anaemia upon the patient’s initial presentation was probably due to the coagulopathy as mentioned, along with the more common finding in celiac disease of iron deficiency anaemia in up to 50% of patients with celiac disease at some point[6]. While gastrointestinal symptoms are common in celiac disease, clinicians should consider celiac disease in patients with unexplained coagulopathies. A gluten-free diet and appropriate supplementation are crucial for management. The response to dietary changes underscores the importance of recognising and treating underlying celiac disease to prevent life-threatening complications.

Acknowledgments

We thank the patient for consenting to the publication of this report, and we thank the entire medical personnel involved in the case.

APPENDIX Other test results

Test	Result	Normal range	
Bilirubin total (mg/dl)	0.4	0.2–1.2	
ALT (U/l)	45 ↑	4–41	
AST (U/l)	33	5–40	
LDH (U/l)	215	135–225	
ALP (U/l)	173 ↑	40–129	
GGT (U/l)	117 ↑	10–55	
Total protein (g/dl)	6.7	6.4–8.3	
Albumin (g/dl)	3.2 ↓	3.4–4.8	
Globulin (g/dl)	3.5	2.2–4	
CRP (mg/l)	279 ↑	≤5.0	
Cholesterol (mg/dl)	54.1	<200	
eGFR (ml/min/1.73m2)	>60	>60	
HS troponin T-B (ng/l)	37	≤14	
CRP-B (mg/l)	54.1 ↑	≤5.0	
Osmolality calculated-B (mOsm/kg)	279 ↓	280–301	

Test	Result	Normal range	
Cardiolipin IgG (U/ml)	2.7	0–10	
ANCA-PR3, Ab-B (U/ml)	2.2	0–10	
Cardiolipin IgM (U/ml)	1.2	0–10	
Beta2-glycoprotein IgG (U/ml)	2.7	0–5	
Beta2-glycoprotein IgM (U/ml)	1.7	0–5	
Anti-nuclear Ab (ANA)	Negative	-	
ANCA-MPO Ab-B (U/ml)	1.1	0–5	

Test	Result	Normal range	
RDW (%)	19.8 ↑	12.0–14.7	
PDW (fl)	12.2	9.5–15.2	
MPV (fl)	10.7	7.3–11.5	
PLT (103/μl)	400	140–450	
NRBC abs. (103/μl)	0.0	0.0	
NRBC (%)	0.0	0.0	
NEU abs. (103/μl)	6.2	2–7.7	
LYM abs. (103/μl)	2.1	1–4	
MONO abs. (103/μl)	0.5	0.2–1.2	
EOS abs. (103/μl)	0.1	0.0–0.7	
BASO abs. (103/μl)	0.0	0.0–0.2	
NEU (%)	69.8	50–70	
LYM (%)	23.4	18–4	
MONO (%)	5.6	2–11	
BASO (%)	0.2	0–2	
EO (%)	1	0–2	
IG (%)	1	0–1	
Fibrinogen (mg/dl)	294.3	170–420	

Test	Result	Normal range	
DRVVT screen (sec)	49.1 ↑	25.7–46.1	
DRVVT confirm (sec)	37.4 ↑	25.4–34.6	
RVVT ratio	1.31	0–1.4	
SCT screen (sec)	58.5 ↑	24–38.8	
SCT confirm (sec)	46.5 ↑	24.2–45.5	
SCT ratio	1.3	0–1.3	

Test	Result (03/12/23 12:40)	Normal range	
Citrated kaolin (CK)-R (min)	10.1 ↑	4.6–9.1	
Citrated kaolin (CK)-K (min)	1.8	0.8–2.1	
Citrated kaolin (CK)-angle (deg)	69.7	63–78	
Citrated kaolin (CK)-MA (mm)	69.3 ↑	52–69	
Citrated kaolin (CK)-LY30 (%)	0.0	0.0–2.6	
Citrated rapid test (CRT)-R (min)	1.5 ↑	0.3–1.1	
Citrated rapid test st (CRT)-K (min)	0.9	0.8–2.7	
Citrated rapid test (CRT)-angle (deg)	76.2	60–78	
Citrated rapid test (CRT)-MA (mm)	70.4 ↑	52–70	
Citrated rapid test (CRT)-LY30 (%)	0.0	0.0–2.2	
aPTT (sec)	48.6 ↑	25–4	
Citrated kaolin heparinase (CKH)-R (min)	13 ↑	4.3–8.3	
Citrated kaolin heparinase (CKH)-K (min)	1.3	0.8–1.9	
Citrated kaolin heparinase (CKH)-angle (deg)	73.1	64–77	
Citrated kaolin heparinase (CKH)-MA (mm)	69.6	52–69	
Citrated functional fibrinogen (CFF)-MA (mm)	34.4	15–32	
PT-SEC (sec)	26.1 ↑	10.03–12.43	
TEG-ACT (sec)	21.9 ↑	82–152	

Figure 1 A chest CT showing bilateral emphysemas and signs indicating diffuse alveolar haemorrhage.

Table 1 Initial laboratory tests.

Test	Result	Normal range	
White blood count (103/μl)	8.9	4–11	
Red blood count (106/μl)	2.7 ↓	4.4–5.9	
Haemoglobin (g/dl)	6.0 ↓	13.5–17.5	
Haematocrit test (%)	18.9 ↓	40–52	
Reticulocytes (%)	1.9 ↑	0.5–1.5	
Reticulocytes (106/μl)	0.05	–	
aPTT (sec)	50.5 ↑	25–40	
Prothrombin time (sec)	75.60 ↑	10.03–12.43	
INR	7.47 ↑	0.97–1.19	
Prothrombin time (%)	6 ↓	75–125	
D-dimer (mg/l FEU)	0.28	0.05–0.55	
Sodium (mmol/l)	132 ↓	135–145	
Potassium (mmol/l)	3.7	3.5–5.1	
Chloride (mmol/l)	96	96–106	
Glucose (mg/dl)	124 ↑	80–115	
Urea (mg/dl)	43.1	20–45	
Creatinine (mg/dl)	0.78	0.7–1.2	
Calcium (mg/dl)	6.9 ↓	8.6–10.3	
Magnesium (mg/dl)	1.6 ↓	1.7–2.5	

Table 2 Iron studies.

Test	Result	Normal range	
Iron (μg/dl)	19.9	59–158	
Transferrin (g/l)	1.8	2–3.6	
Transferrin saturation (%)	7.9	15–45	
Ferritin (μg/l)	228	30–400	
Total iron-binding capacity (μg/dl)	251	250–400	
Unsaturated iron-binding capacity (μg/dl)	231	120–470	
Vitamin B12 (pg/ml)	616	200–770	
Folic acid (ng/ml)	4.6	4.6–18.7	

Table 3 INR values throughout the first nine days of hospitalisation.

INR values	
Day 0	Day 1	Day 2	Day 3	Day 5	Day 6	Day 7	Day 8	Day 9	
7.47	3.47	2.07	2.48	1.19	1.11	1.47	2.35	3.1	

Table 4 Celiac and vitamins studies.

Test	Result	Normal range	
Anti-tissue transglutaminase IgA-B (U/ml)	104 ↑	0–10	
IgA (celiac)-B	464.2 ↑	70–−400	
25 hydroxyvitamin D (ng/ml)	4 (deficiency)	-	
Thiamine (vitamin B1) (mcg/l)	82 ↑	33–1–60.7	
Tocopherol (vitamin E) (mg/l)	2 ↓	5–18	
Retinol (vitamin A1) (μg/dl)	6 ↓	30–70	

Conflicts of Interests: The Authors declare that there are no competing interests.

Patient Consent: Written informed consent was obtained from the patient for publication of this report.
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