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British Journal of Healthcare and Medical Research - Vol. 11, No. 3
Publication Date: June 25, 2024
DOI:10.14738/bjhmr.113.16929.
Sanchez, O. N. M., Valerio, M. G. P., Manrique, A. C., García, C. L., Garza, J. T., & Cantú, L. A. M. (2024). The Role of the Surgeon in
Primary Immune Thrombocytopenia: Case Report. British Journal of Healthcare and Medical Research, Vol - 11(3). 155-160.
Services for Science and Education – United Kingdom
The Role of the Surgeon in Primary Immune Thrombocytopenia:
Case Report
Olga Nidia Montero Sánchez
Posgrado de cirugía general en Hospital General de Saltillo, Coahuila. México
María Gabriela Pesqueira Valerio
Posgrado de cirugía general en Hospital General de Saltillo, Coahuila. México
Antonio Camacho Manrique
Especialidad cirugía general en Hospital General de Saltillo, Coahuila. México
Carlos Lizcano García
Especialidad cirugía general en Hospital General de Saltillo, Coahuila, México
Jessica Torres Garza
Posgrado de cirugía general en Hospital General de Saltillo, Coahuila. México
Luis Alfonso Morales Cantú
Posgrado de cirugía general en Hospital General de Saltillo, Coahuila. México
ABSTRACT
Idiopathic thrombocytopenic purpura (ITP) is important as it is one of the most
common thrombocytopenias in adults. Despite their medical treatment, there is a
percentage of refractory patients who persist with severe hemorrhages, requiring
splenectomy; this is where the surgeon becomes involved in this condition. The
objective of this work is to present our experience with this disease and
laparoscopic splenectomy in the general surgery service of the General Hospital of
Saltillo, Coahuila, Mexico. We present the case of a 40-year-old male with a diagnosis
of ITP since he was 28 years old, who for the last 12 years presented recurrent
symptoms of severe hemorrhagic events with partial and temporary improvement
of the condition despite aggressive pharmacological treatment. We were requested
to consult the general surgery service due to therapeutic failure of pharmacological
treatment, and a protocol for laparoscopic splenectomy was initiated. The patient
progressed adequately and was discharged after the procedure with follow-up by
outpatient clinic with a biopsy negative for malignancy.
Keywords: Idiopathic thrombocytopenic purpura, Splenectomy, laparoscopy,
Introducción
INTRODUCTION
Immune diseases are multifactorial and poorly understood thanks to their great diversity of
both clinical and laboratory manifestations. Primary immune purpura (previously called
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immune thrombocytopenic purpura),1 characterized by being an isolated thrombocytopenia
with a platelet count <100 x 109/l without apparent cause, is no exception to this statement.2
The majority of patients may have an asymptomatic course, or present minimal manifestations
of the disease, however, approximately 5-6% may trigger severe bleeding.
The initial treatment of ITP is pharmacological. However, when these resources fail, we resort
to surgical treatment. Splenectomy corresponds to a second-line medical indication, indicated
mainly in older patient’s refractory to first-line medical therapy. This is where the role of the
surgeon is fundamental, but when to perform it? Under what conditions? How many platelets
is it safe to perform the procedure with?
Next, we present the pre-trans and post-surgical management of a 40-year-old male patient
with ITP refractory to medical treatment.
CASE PRESENTATION
A 40-year-old male with no significant family history, has difficult-to-control systemic arterial
hypertension diagnosed 6 months ago and currently being managed with telmisartan,
bisoprolol and nifedipine. Patient presents as a smoker with a smoking index (TI) of 2.2
packs/year.
He has been diagnosed with ITP since he was 28 years old. During the last 12 years, the patient
has presented recurrent symptoms of severe hemorrhagic events such as severe epistaxis,
hematomas in soft tissues and hyposphagma associated with thrombocytopenia that has
required transfusions. Managed with danazol, prednisone, mycophenolate acid, rituximab,
platelet apheresis and erythrocyte concentrates, he has remained under follow-up in the
hematology service, however, with partial and temporary improvement of the condition
despite aggressive pharmacological treatment.
We were requested to consult the general surgery service due to therapeutic failure of
pharmacological treatment, and a protocol for splenectomy was initiated.
At the time of evaluation, the patient was asymptomatic, with the last severe epistaxis a month
ago.
Patient comes from the hematology clinic with complete vaccination for splenectomized
patients. Anti-pneumococcal, anti-meningococcal and haemophilus influenzae type B vaccines
are administered.
In addition to multiple studies in search of probable etiology of ITP (Table 1):
Table 1
Study Result
Hemoglobin 15.4g/dL
Leukocytes 7.9 10e3 /uL
Platelets 14,000 10e3/uL
TP 13.0 sec
TPT 71.9 sec
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Sanchez, O. N. M., Valerio, M. G. P., Manrique, A. C., García, C. L., Garza, J. T., & Cantú, L. A. M. (2024). The Role of the Surgeon in Primary Immune
Thrombocytopenia: Case Report. British Journal of Healthcare and Medical Research, Vol - 11(3). 155-160.
URL: http://dx.doi.org/10.14738/bjhmr.113.16929.
INR 1.07
Anti-cytoplasmic antibodies (C-ANCA) 0.40 UR/mL
Anti-cystoplasmic antibodies (P-ANCA) 0.68 UR/mL
Hepatitis B Negative
Hepatitis C Negative
Anti Helicobacter pylory antibodies IGG 60.3 UR/mL
HIV Non reactive
Imaging studies are also performed, reporting the following in Doppler ultrasound:
• Hepatic steatosis grade II
• Splenomegaly 12.8cm
• Normal portal venous system, splenic vein and hepatic artery.
Without relevant findings, we proceeded to follow the surgical protocol for laparoscopic
splenectomy. He entered the operating room with a total of 30,000 platelets. (Fig. 1 and 2).
Patient progresses adequately and is discharged after the procedure with follow-up by
outpatient clinic with biopsy negative for malignancy.
Figure 1 and 2. Spleen measuring 13 x 11 x 4.5 cm Weight 300g, Minimally invasive incisions
with Pfannenstiel incision
DISCUSSION
ITP is a disease characterized by an isolated decrease in the number of platelets below
100,000/mm3, without finding a triggering cause to explain it.1,2
Classification criteria based on duration were revised, so chronic ITP was defined as disease
lasting more than 12 months.3
ITP has an incidence of 2-4 per 100,000 adults each year and a prevalence of 9-20 per 1000,000
adults, being more common in women, reaching gender equality at the age of 60 years.4
Regarding its pathophysiology, platelet destruction by antibodies is the canonical mechanism
of platelet destruction in ITP. Although approximately 20 to 40% of ITP patients have no
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detectable anti-GP autoantibodies, suggesting alternative mechanisms of platelet destruction.
Different possible mechanisms by which thrombocytopenia occurs have been described, some
of them are the following:1
• Antiplatelet glycoprotein antibodies (mainly, II b/III a)
• Cytotoxicity by CD8+ T cells.
• Mechanisms that act on megakaryocytes and inhibit platelet synthesis.
• Inadequate levels of thrombopoietin contribute to this decreased synthesis.
Most patients are asymptomatic or have only mild mucocutaneous hemorrhage; however,
serious bleeding occurs in 5 to 6% of patients.2
Updated American Society of Hematology (ASH) guidelines recommend initiation of disease- specific treatments if platelet counts are <30 × 109/L, through medical treatment.6
Regarding the initial treatment of choice, corticosteroids such as dexamethasone and
prednisone are used. Approximately 40% of patients will respond to this measure. Other
treatments such as intravenous anti-RhD, rituximab show partial improvement. As alternatives
for those who do not respond to corticosteroids, other drugs such as Romiplostim and
eltrombopag can be used, recently developed drugs that mimic the action of
thrombopoietin.6,7,8
Emergency treatment will be assessed according to the hemorrhagic events and the
hemodynamic stability of the patient, requiring combined pharmacological treatment and
transfusion of erythrocyte and platelet concentrates.
And as a second-line therapy in the absence of response to medical treatment, the surgeon's
intervention with a splenectomy is the most effective therapy in patient’s refractory to
treatment, with multiple bleeding events.7
According to the ASH, surgical treatment can be performed laparoscopically or openly. I
consider laparoscopy to be the method of choice in spleens <20 cm due to the already known
advantages of minimally invasive surgery, such as rapid recovery, less pain, less days of hospital
stay. It is recommended to wait 12 months after the diagnosis of ITP due to the high rate of
disease regression during the first year, as well as the administration of vaccines against
encapsulated organisms (Streptococcus pneumoniae, Neisseria meningitidis and Haemophilus
infuenzae) at least 2 weeks before splenectomy.6 Regarding the platelet count, it is safe to
perform the procedure with a platelet count equal to or > 30,000, and if a count < 30,000 is
present, platelet apheresis transfusions may be performed during the procedure, at the time of
clamping the splenic vessels. It is important to emphasize that having a count lower than
suggested should not be an impediment or contraindication to performing the procedure, since
in these patients we will not be able to maintain normal platelets.15
CONCLUSION
The role of the surgeon in ITP is of utmost importance in patient’s refractory to treatment,
associated with persistent symptoms.
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Sanchez, O. N. M., Valerio, M. G. P., Manrique, A. C., García, C. L., Garza, J. T., & Cantú, L. A. M. (2024). The Role of the Surgeon in Primary Immune
Thrombocytopenia: Case Report. British Journal of Healthcare and Medical Research, Vol - 11(3). 155-160.
URL: http://dx.doi.org/10.14738/bjhmr.113.16929.
We should not postpone surgical treatment waiting to find an ideal platelet level, since this can
hardly be achieved. The procedure can be performed with a platelet count >30,000 and platelet
apheresis can be used for the trans-surgical procedure.
Interest conflict
We declare ourselves without conflict of interest
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