A rare case of spontaneous haemothorax in patient with haemophilia A

Abstract

Haemorrhage in patients with haemophilia is common after minor trauma but may occur spontaneously. Despite the diversity of bleeding sites, spontaneous haemothorax, on a non-traumatic basis, is an exceedingly rare event and only a few cases had been reported. We present a case of a 43-year-old man with a history of haemophilia A who had pleuritic chest pain for 1 day without significant history of trauma. Diagnostic thoracentesis showed bloody pleural fluid in which neither abnormal cell nor organism was found. He was treated by cryoprecipitate replacement and therapeutic thoracentesis for releasing haemothorax. After discharge, the patient returned for follow-up with complete radiological resolution. Regarding the consequences of retained haemothorax from conservative approach and the procedure-related bleeding of given therapeutic intervention in haemothorax making its management in patients with haemophilia to be more challenging. Our case illustrates a conservative treatment of spontaneous haemothorax in patient with haemophilia resulting in a good clinical outcome.

Background

Patients with haemophilia often encounter haemorrhagic episodes which could be either trauma-related or spontaneous bleeding. Bleeding in joints and muscles of the extremities are common that could be easily controlled with factor replacement. However, spontaneous bleeding in uncommon sites, for example, internal organ bleeding, haemothorax, hemoperitoneum, had been reported with catastrophic consequences if the bleeding was uncontrolled. We report a case of spontaneous haemothorax in a patient with haemophilia that could be successfully managed by cryoprecipitate administration and therapeutic thoracentesis.

Case presentation

A 43‐year‐old man presented to the emergency department with sudden left-sided pleuritic chest pain for 1 day. He denied cough, fever or shortness of breath. His medical history included haemophilia A with multiple episodes of spontaneous haemarthrosis and a history of blunt abdominal injury with internal organ bleeding. Factor VIII inhibitor was periodically screened with the last value of 0.73 Bethesda unit (BU) 1 month prior to this admission.

Physical examinations revealed stable vital signs with decreased breath sounds and dullness on percussion at the left lower lung base. Initial laboratory studies were as follows; haemoglobin 129 g/L, haematocrit 40.1%, platelet 304 x10⁹/L, white cell count 14 030/μL (neutrophils 76.7%, lymphocytes 17.7% and monocytes 5.1%), activated partial thromboplastin time (APTT) 52.7 s (control 25.5), international normalized ratio (INR) 1.03. Factor VIII inhibitor level was 0.54 BU. The X-ray of the chest was consistent with left pleural effusion (figure 1).

Figure 1.

Chest radiographs on initial presentation (A), before therapeutic thoracentesis was performed (B), after releasing 700 mL of haemothorax (C) and 10 months post-treatment.

After 2 weeks, he was admitted to the hospital as the X-ray of the chest showed increased amount of left pleural effusion (figure 1). Fifteen units of cryoprecipitate were given followed by diagnostic thoracentesis which yielded 75 mL of unclotted blood. The pleural fluid analysis included haematocrit 20% (pleural fluid/serum haematocrit of 50%), white blood cells 1217/μL (PMN 25.2% and lymphocyte 74.8%) and red blood cells 2.49x10¹²/L. Pleural fluid for mycobacterial culture, PCR for Mycobacterium tuberculosis and cytology were all negative.

Cryoprecipitate was given for maintaining 100% of factor VIII activity level. Therapeutic thoracentesis yielded 700 mL of unclotted blood. The X-ray of the chest after thoracentesis revealed a significant reduction of haemothorax (figure 1). Correction of factor VIII activity level by cryoprecipitate was gradually tapered down from 100% to 20% in 12 days. Neither recurrence of haemothorax nor complication from retained clot was observed by pleural ultrasonography and X-ray of the chest. He was discharged without requiring any further intervention.

Outcome and follow-up

Neither recurrence of left pleural effusion nor clinical symptom of pleurisy was observed on 10-month follow-up (figure 1).

Discussion

Spontaneous haemothorax in patients with haemophilia is distinctively rare of which 10 cases of children and adults had been reported in the literature to date^1–8 (table 1). Of these, three were reported to have the high titre of factor VIII inhibitors level which is prevalent in patients with haemophilia with prior history of factor VIII replacement.^4–6 Interestingly, a diagnosis of spontaneous haemothorax was made in our patient, in particular, a low level of factor VIII inhibitor was observed.

Table 1.

Summary of patients with haemophilia with spontaneous haemothorax in the literature review.

Author	Publication year	Age	Presentation	Treatment	Outcome	X-ray of the chest on follow-up
Kay and Kupfer2	1957	20	Chest pain, dyspnoea	Lyophilised antihaemophilic plasma Thoracentesis	Alive	Residual pleural thickening
Barrett and Israels7	1965	Not mentioned	Chest pain, dyspnoea	Observation	Alive	Complete resolution
		18	Chest pain, dyspnoea	Antihaemophilic globulin	Alive	Partial resolution
Bart1	1972	17	Chest pain	Cryoprecipitate	Alive	Complete resolution
Rasaretnam et al8	1976	27	Chest tightness and pain	Blood transfusion Thoracentesis	Alive	Nearly complete resolution
Wilimas et al3	1985	7	Chest pain, dyspnoea	Factor VIII replacement Thoracentesis	Alive	Complete resolution
		15	Chest pain, dyspnoea	Factor VIII replacement	Alive	Not mentioned
Gidaris et al4	2010	6	Chest pain	Recombinant factor VIIa	Alive	Not mentioned
Obitko-Pludowska et al5	2010	15	Fever, backache	Recombinant factor VIIa and factor eight inhibitor bypassing activity (FEIBA) Thoracentesis	Alive	Complete resolution
Peyman Eshghi et al6	2019	12	Chest pain	Recombinant factor VIIa and FEIBA Thoracentesis Open thoracotomy	Dead	None

Practically, haemothorax should be managed by tube thoracostomy aiming to assess the rates of blood loss and prevent complications caused by organising clot. The indications for surgical exploration in haemothorax are massive haemothorax, continued bleeding and haemodynamic instability.⁹ Given the risk of procedure-related bleeding in patient with haemophilia, the management of any haemorrhagic episode is likely to be more conservative than in patient with non-haemophilia. However, there is no consensus about the treatment of haemothorax in patients with haemophilia but to achieve haemostasis with bypassing agent or factor replacement may be preferable to surgical intervention.

Conservative management in spontaneous haemothorax result in the good clinical outcomes as demonstrated by several reports. Barrett and Israels reported two patients with haemophilia with spontaneous haemothorax who had been treated conservatively by anti-haemophiliac globulin or fresh plasma replacement resulting in incomplete lung expansion in one patient.⁷ In 1985, conservative treatment with factor VIII concentrate replacement had been later proved to be an effective treatment as Wilimas et al reported in three patients with haemophilia diagnosed with haemothorax and hemomediastinum. None of them required any intervention and the bleeding was also completely subsided. They speculated that, in patients with haemophilia, organising clot could be less likely to develop because of low levels of factor VIII in haemothorax.³

To date, the novel bypassing agents such as recombinant activated factor VII and factor eight inhibitor bypassing activity are available for control of bleeding in patients with haemophilia. Their efficacy in treating those with spontaneous haemothorax was demonstrated in some case reports.^{4 5} On the other hand, despite receiving these novel agents, there were several cases that had the detrimental outcome resulting in clotted haemothorax requiring thoracotomy and decortication.⁶ However, due to limited case reports, the method of draining haemothorax in a patient with haemophilia is still a controversial issue. We believe that the timing and type of intervention needed to be determined based on several factors for example, haemodynamics, the severity of coagulation defect. However, balancing between consequent complications from a conservative approach and bleeding complications from aggressive procedures is the important considering aspect in such a case. As our patient, concerning about the complications of retained haemothorax and potential risk of bleeding from an invasive procedure, we decide to administer cryoprecipitate to achieve haemostasis and perform therapeutic thoracentesis afterwards.

In conclusion, we report a patient with haemophilia with spontaneous haemothorax successfully treated by cryoprecipitate replacement and therapeutic thoracentesis which could achieve haemostasis and show no consequence of clotted haemothorax. Hence, in hereditary bleeding disorder who are at high risk of procedure-related bleeding complications, conservative management and avoiding such an aggressive intervention should be considered.

Learning points.

• Spontaneous haemothorax is rare in haemophilia, particularly in mild severity.

• The mainstay treatment in haemothorax, in general, is tube thoracostomy and surgical management if indicated. However, in patients with haemophilia, less invasive or conservative management might be preferable regarding the bleeding consequences related to the procedure.

• The complications from retained haemothorax which may attribute to the worse outcomes should be also concerned.