Splenectomy in patients with chronic myelomonocytic leukemia: Indications, histopathological findings and clinical outcomes in a single institutional series of thirty‐nine patients

Abstract

In a 28-year period, 39 (7%) patients with chronic myelomonocytic leukemia (CMML) (median age 66 years, 64% male) underwent a splenectomy at our institution. Primary indications for splenectomy were refractory thrombocytopenia (36%), progressive spleen related symptoms (33%), emergent splenectomy for splenic rupture (21%), refractory anemia (8%), and prior to allogeneic stem cell transplant (3%). Eleven (28%) patients had anemia at the time of splenectomy, of which 3 (27%) were autoimmune. The median time to splenectomy from CMML diagnosis was 6 months (0-40); perioperative morbidity and mortality rates were 43% and 13%, while the median postsplenectomy survival was 25 months (11-38). Durable remission in spleen related symptoms, thrombocytopenia, complications from splenic rupture, and anemia were achieved in 85%, 50%, 62%, and 21% of patients, respectively. Perioperative morbidity (n = 30) included infections/sepsis in 6 (20%), intraabdominal bleeding in 4 (13%), venous thromboembolism (VTE) in 3 (10%), and acute lung injury in 2 (7%) patients. The median duration of hospital stay was 6 days (1-25), with 5 deaths occurring secondary to respiratory failure (n = 2), multiorgan dysfunction (n = 2) and hemorrhagic shock (n = 1). There was no difference in overall survival between CMML patients that underwent splenectomy, in comparison to those that did not. Unlike in myelofibrosis, portal hypertension was not an indication for splenectomy and no patients developed post-splenectomy thrombocytosis. In conclusion, apart from being a lifesaving emergent modality in the event of splenic rupture, splenectomy has an important palliative role in patients with CMML, with significant and durable improvements in spleen related symptoms and refractory cytopenias.

Peliosis lienalis with atraumatic splenic rupture in a patient with chronic myelomonocytic leukemia: A case report

“…However, the rate of atraumatic splenic rupture in CMML is low and described in case reports [ 4 , 18 , 19 ]. A retrospective study of 555 patients with CMML demonstrated a 7% rate of splenectomy in the time period from 1980 until 2018 [ 20 ]. In 8 (21%) patients the indication was a splenic rupture with six atraumatic and two traumatic ruptures.…”

How I diagnose and treat chronic myelomonocytic leukemia

“…The median duration of ESA response is 12-18 months, with limited options after progression. I usually use fixed doses of recombinant human erythropoietin or darbepoetin and strictly avoid the use of G-CSF (granulocyte-colony stimulating factor) given the higher baseline risk for splenic rupture in CMML patients 73 . I closely monitor for adverse vascular side effects associated with ESAs' such as treatment emergent hypertension and thromboembolism and do not administer these agents when the HB levels are >11 gm/dl.…”

“…Clinical issues related to splenomegaly include early satiety, abdominal pain and tenderness, constitutional symptoms, referred shoulder pain, hiccoughs and mechanical obstruction of abdominal organs 77 . Splenic infarction and spontaneous splenic rupture can result in abdominal catastrophes 73 . We usually manage symptomatic splenomegaly, or massive splenomegaly, with cytoreductive therapy, with hydroxyurea being our first choice.…”

“…I usually use fixed doses of recombinant human erythropoietin or darbepoetin and strictly avoid the use of granulocyte – colony-stimulating factor) given the higher baseline risk of splenic rupture in CMML patients. 73 I closely monitor for adverse vascular side effects associated with erythropoiesis-stimulating agents, such as treatment-emergent hypertension and thromboembolism and do not administer these agents when hemoglobin levels are >11 g/dL. Luspatercept is a recombinant fusion protein that traps GDF 11 and activin ligands belonging to the TGF-β superfamily, decreasing SMAD2 and SMAD3 signaling, enabling latestage erythroid maturation and has been approved by the Food and Drug Administration for patients with β-thalassemia and MDS-ring sideroblasts.…”

Advances in chronic myelomonocytic leukemia and future prospects: Lessons learned from precision genomics

“…Similar to MDS, other palliative interventions include hydroxyurea for patients with proliferative CMML, use of erythropoietin analogues, prophylactic antibiotics for persistent neutropenia, splenectomy for persistent splenomegaly‐related symptoms, and iron chelators for patients dependent on RBC transfusions . No CMML‐specific guidelines exist on how to utilize these palliative treatments and should be recommended for patients at the discretion of the treating hematologist.…”