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Gray Platelet Syndrome and Total Abdominal Hysterectomy: Perioperative and Anaesthetic Management of the First Reported Case in Mexico

This paper reports the first documented case in Mexico of a 32-year-old woman with Gray Platelet Syndrome successfully undergoing a total abdominal hysterectomy through multidisciplinary perioperative management, including platelet transfusion, antifibrinolytic therapy, and balanced general anesthesia, which controlled significant intraoperative bleeding without causing metabolic decompensation.

Original authors: Martha Rocio Duran-Badillo, Mirna Magali Delgado-Carlo

Published 2026-07-07

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CC BY 4.0

Original authors: Martha Rocio Duran-Badillo, Mirna Magali Delgado-Carlo

Original paper licensed under CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). ✨ This is an AI-generated explanation of the paper below. It is not written or endorsed by the authors. For technical accuracy, refer to the original paper. Read full disclaimer

The Big Picture: A Rare "Glitch" in the Blood

Imagine your body's blood is a busy construction site. When you get a cut, your body sends in "repair crews" (platelets) to patch the hole and stop the bleeding.

In most people, these crews are well-equipped with tools (proteins in granules) to do their job. But this patient had Gray Platelet Syndrome (GPS). Think of her repair crews as arriving at the construction site with empty toolboxes. They are there in decent numbers, but they lack the essential tools (specifically, alpha-granules) needed to actually fix the leak.

This is a very rare condition (fewer than 200 cases worldwide). Because the crews look like they are there, doctors often get tricked. This patient was previously misdiagnosed with a different condition (ITP) and treated with steroids that didn't really help. It wasn't until a high-tech microscope (electron microscopy) looked inside the "toolboxes" and saw they were empty that the real diagnosis was made.

The Challenge: A Big Surgery with a Leaky System

The patient, a 32-year-old woman, needed a total abdominal hysterectomy (removal of the uterus) because of fibroids causing heavy bleeding.

The Dilemma:

The Risk: Surgery is risky for her because her "repair crews" are defective. Even if the number of crews looks okay, they can't stop the bleeding effectively.
The Anesthesia Trap: Usually, for this type of surgery, doctors might use a "spinal block" (numbing the lower body while the patient is awake). However, this is like trying to park a car in a garage with a cracked foundation. If the blood can't clot properly, a spinal block could cause a dangerous bleed inside the spine. The paper states this was strictly forbidden for her, regardless of her platelet count.

The Game Plan: The "Pre-Game" Prep

Before the surgery, the medical team (doctors, blood specialists, and anesthesiologists) worked together for 72 hours to get her ready.

Boosting the Numbers: They gave her a special blood transfusion (apheresis platelets) and immune globulin (IVIG). This was like hiring a massive fleet of extra repair crews to ensure there were enough bodies on the site, even if their toolboxes were still a bit light.
The "Anti-Leak" Shield: They decided to use Tranexamic Acid. Think of this as a super-strong "glue" or "sealant" that helps the blood clot faster, acting as a backup plan since the crews' natural tools were missing.
The Anesthesia Choice: They chose General Anesthesia (putting her to sleep). They picked specific drugs (like Fentanyl and Propofol) that act like a gentle, steady hand—calming the body without messing up the blood's ability to clot.

The Surgery: A Close Call, But a Win

During the 3-hour and 40-minute surgery, things went smoothly at first. But once the uterus was removed, the bleeding increased.

The Crisis: They estimated about 400 mL of blood loss right then.
The Response: The team immediately gave more of the "sealant" (Tranexamic acid) and some calcium (to help the clotting process work).
The Result: The bleeding stopped. The total blood loss for the whole surgery was about 900 mL (roughly two large bottles of water). While this is a lot, her body handled it well. Her blood pressure stayed stable, and her organs didn't suffer from lack of oxygen.

The Aftermath

After the surgery, her blood count dropped a little, so they gave her one unit of red blood cells to top her up. She woke up, was stable, and was sent to the regular ward.

Key Takeaways from the Paper

Count Doesn't Equal Safety: Just because you have a "good number" of platelets doesn't mean you are safe from bleeding if the platelets are defective. It's like having 100 firefighters who all forgot their hoses; the number is high, but the fire won't go out.
No Spinal Blocks: For GPS patients, you cannot use spinal anesthesia because the risk of a spine bleed is too high, even if the platelet count looks okay.
The "Sealant" Strategy: Using anti-fibrinolytic drugs (like Tranexamic acid) is a crucial tool to stop bleeding when the body's natural clotting is broken.
Teamwork Wins: This was the first time this specific condition was managed with anesthesia in Mexico. The success came from a team of specialists planning ahead, optimizing the patient's blood before surgery, and being ready to act fast when bleeding started.

In short: This paper tells the story of how a rare blood disorder was successfully managed during major surgery by recognizing the hidden defect, avoiding dangerous anesthesia techniques, and using a "glue" strategy to stop the bleeding, all while keeping the patient stable.

Technical Summary: Perioperative and Anaesthetic Management of Gray Platelet Syndrome in a Total Abdominal Hysterectomy

Problem Statement
Gray Platelet Syndrome (GPS) is an exceptionally rare inherited disorder characterized by platelet $\alpha$ -granule deficiency, leading to thrombocytopenia and qualitative platelet dysfunction. The condition presents a significant perioperative challenge due to an unpredictable hemorrhagic risk that is often dissociated from the absolute platelet count. Patients are frequently misdiagnosed as having immune thrombocytopenia (ITP), leading to inappropriate immunosuppressive therapy and unmanaged surgical risks. Prior to this report, no case of GPS managed anaesthesiologically had been documented in Mexico, and literature regarding the specific anesthetic and surgical management of this condition in the Latin American context remains critically sparse.

Methodology
This case report details the perioperative management of a 32-year-old female with a confirmed diagnosis of GPS (established via platelet aggregometry and transmission electron microscopy showing marked $\alpha$ -granule reduction) who required a total abdominal hysterectomy for uterine myomatosis and abnormal uterine bleeding.

The management strategy involved a multidisciplinary approach (hematology, gynecology, and anesthesiology) focusing on:

Preoperative Optimization: Over a 72-hour period, the patient received apheresis platelet transfusions and intravenous immunoglobulin G (IVIg) to raise the platelet count from a baseline of 41,000/µL to 80,000/µL.
Anesthetic Technique Selection: Neuraxial blockade was explicitly contraindicated due to the qualitative platelet defect, regardless of the platelet count. Balanced general anesthesia was selected.
Pharmacological Strategy:
- Agents Used: Induction and maintenance utilized fentanyl, propofol, vecuronium, and sevoflurane. Adjuvants included lidocaine, paracetamol, dexamethasone, dexmedetomidine, ondansetron, and furosemide.
- Agents Avoided: Substances known to impair platelet function (NSAIDs, semi-synthetic penicillins, phenothiazines, hydroxyethyl starch) were strictly avoided.
- Hemostatic Support: Prophylactic tranexamic acid (1 g IV) was administered at induction. Desmopressin (DDAVP) was withheld due to inconsistent evidence of efficacy in GPS.
Monitoring: Serial arterial blood gas (ABG) analyses were performed intraoperatively and in the post-anaesthesia care unit (PACU) to monitor for metabolic acidosis and tissue hypoperfusion.

Results
The patient underwent a 3-hour and 40-minute procedure with the following outcomes:

Hemorrhage: Total intraoperative blood loss was approximately 900 mL. A significant increase in bleeding (estimated 400 mL) occurred immediately following specimen removal, prompting the administration of rescue tranexamic acid (1 g) and calcium gluconate (1 g).
Hemodynamic Stability: The patient remained hemodynamically stable throughout. No vasopressors were required. Serial ABG analyses confirmed the absence of metabolic acidosis or significant tissue hypoperfusion despite the blood loss.
Transfusion Requirements: No intraoperative red blood cell transfusion was necessary. In the PACU, hemoglobin declined to 9.8 g/dL, prompting the transfusion of one unit of packed red cells, which was tolerated without adverse reaction.
Postoperative Course: The patient was discharged to the ward hemodynamically stable with an Aldrete score of 10 and no immediate anesthetic complications.

Key Contributions

First Reported Case in Mexico: This document represents the first reported instance of GPS managed anaesthesiologically in Mexico, adding to the very limited global literature (fewer than 200 cases worldwide).
Validation of General Anesthesia: The case reinforces that general anesthesia is the technique of choice for GPS, while neuraxial blockade remains contraindicated due to the risk of spinal hematoma, even when platelet counts appear adequate.
Dissociation of Count and Function: The case empirically demonstrates that platelet count is an unreliable predictor of hemorrhagic severity in GPS. Despite optimization to 80,000/µL, significant bleeding occurred, highlighting the dominance of the qualitative $\alpha$ -granule defect.
Efficacy of Anti-fibrinolytic Strategy: The successful management of intraoperative bleeding using prophylactic and rescue tranexamic acid, without the need for massive transfusion or vasopressor support, supports the use of anti-fibrinolytic therapy as a central pillar in GPS management.
Systemic Phenotype Recognition: The report contextualizes the patient's leukopenia, hypogammaglobulinemia, and chest radiography findings (diffuse interstitial pattern) within the broader systemic phenotype of GPS, linking them to the underlying NBEAL2 mutation and associated immune dysregulation.

Significance and Claims
The authors claim that this case contributes to the "critically sparse perioperative literature" on GPS. The significance lies in demonstrating that favorable surgical outcomes are achievable in this high-risk population through:

Individualized, multidisciplinary planning.
Intensive preoperative optimization (transfusion and IVIg).
Strict avoidance of platelet-function-impairing agents.
The selection of general anesthesia over neuraxial techniques.
Prompt deployment of anti-fibrinolytic therapy and close metabolic monitoring.

The paper concludes that while GPS presents an unpredictable hemorrhagic risk independent of platelet count, a strategy grounded in the pathophysiology of the disease—specifically the qualitative defect—allows for the safe management of major surgical procedures. The authors emphasize that in the absence of curative therapy, management must remain centered on individualized supportive strategies and complication prevention.