many of the hemolytic anemias and platelet disorders respond to splenectomy after failure of medical therapy .
removal of the spleen can play an important role in reducing the mortality of hematologic conditions .
diagnostic splenectomy , while there is no enough data , is still performed in the general surgery . however , there is no indication in gaucher disease for diagnostic splenectomy .
the differential diagnosis of the gaucher disease is considered in patients with unexplained organomegaly , easy bruise , bone pain , or a combination of these conditions .
all suspected diagnoses are confirmed by the determination of the acid - glucosidase activity in isolated leukocytes or cultured fibroblasts .
we hereby report the four patients , three of whom were easily diagnosed gaucher 's disease with classical methods using bone marrow aspiration cytology , and one underwent teh splenectomy due to splenic mass and wasdiagnosed gaucher 's disease after pathological examination .
a 39-year - old woman who intermittently suffered from nasal bleeding lasting 3 days was admitted to our hematology department .
her vital signs on admission were as follows : blood pressure was 110/75 mmhg ; pulse rate was 72 per minute ; and temperature was 36.8 c .
the peripheral blood count revealed a hemoglobin level of 10.5 g / dl , hematocrite value of 33.0% , white blood cell count of 7000/mm3 and trombocyte count was 50,000/mm .
examination of the bone marrow cytology showed gaucher cells . upon this , we diagnosed gaucher and supported our diagnosis with - glucosidase activity in isolated leukocytes or cultured fibroblasts .
a 42-year - old woman who complained of fatigue lasting 4 months was admitted to our internal medicine department .
her laboratory examination revealed a hemoglobin level of 11.5 g / dl , a hematocrite value of 34.0% , a white blood cell count of 9000/mm3 and a trombocyte count of 60,000/mm .
then , we diagnosed gaucher 's disease . a 51-year - old man with the complaints of a 10-day history of fatigue and rectal bleeding admitted to our emergency department .
the laboratory test revealed pancytopenia . under the light of all these findings , we performed bone marrow examination , and gaucher was diagnosed . a 75-year - old man with lower back pain lasting for 2 months admitted to our orthopedic clinic . on physical examination ,
the abdominal computed tomography scan ( ct ) showed hepatomegaly , splenomegaly of 14 cm in width and 4 cm mass at spleen ( fig .
afterwards , we obtained findings of gaucher 's disease ( figs 2 , 3 ) .
bone marrow aspirate with hematoxylen and eosin stain , showing the curtain like macrophages known as gaucher 's cells .
a 39-year - old woman who intermittently suffered from nasal bleeding lasting 3 days was admitted to our hematology department .
her vital signs on admission were as follows : blood pressure was 110/75 mmhg ; pulse rate was 72 per minute ; and temperature was 36.8 c .
the peripheral blood count revealed a hemoglobin level of 10.5 g / dl , hematocrite value of 33.0% , white blood cell count of 7000/mm3 and trombocyte count was 50,000/mm .
examination of the bone marrow cytology showed gaucher cells . upon this , we diagnosed gaucher and supported our diagnosis with - glucosidase activity in isolated leukocytes or cultured fibroblasts .
a 42-year - old woman who complained of fatigue lasting 4 months was admitted to our internal medicine department . after her physical examination , we just found hepatosplenomegaly .
her laboratory examination revealed a hemoglobin level of 11.5 g / dl , a hematocrite value of 34.0% , a white blood cell count of 9000/mm3 and a trombocyte count of 60,000/mm .
a 51-year - old man with the complaints of a 10-day history of fatigue and rectal bleeding admitted to our emergency department .
the laboratory test revealed pancytopenia . under the light of all these findings , we performed bone marrow examination , and gaucher was diagnosed .
a 75-year - old man with lower back pain lasting for 2 months admitted to our orthopedic clinic . on physical examination , a slight hepatosplenomegaly was found . there were not any palpable lymphadenopathy .
the abdominal computed tomography scan ( ct ) showed hepatomegaly , splenomegaly of 14 cm in width and 4 cm mass at spleen ( fig .
afterwards , we obtained findings of gaucher 's disease ( figs 2 , 3 ) .
bone marrow aspirate with hematoxylen and eosin stain , showing the curtain like macrophages known as gaucher 's cells .
ther is no enough data in the literature about diagnostic splenectomy , however in some uncleared conditions ( especially in hematologic disorders ) many surgeons have to perform this procedure throughout their lives . on the other hand , therapeutic splenectomy today is performed in various cases .
although splenectomy does not alter the course of a patient with gaucher 's disease , it is the procedure of choice if there are signs of hypersplenism .
some authors have proposed performing a partial splenectomy for hypersplenism in gaucher 's disease to limit the hypersplenism while preserving some splenic functions .
there are three different recognized types of gaucher 's disease , which are differentiated from each other depending on the presence or absence of neurological symptoms[45 ] .
the first symptom occur before 10 years of age in more than 50% of the patients
. early onset of the clinical symptoms and signs predispose patients to severe phenotype and irreversible complications .
the most prevalent variant of the disease is the non - neuronopathic form ( type 1 gaucher 's disease , or the so - called 
patients in this group usually bruise easily and experience fatigue due to anemia and low blood platelets .
they also have an enlarged liver and spleen , skeletal disorders , and , in some instances , lung and kidney impairment .
patients have extensive and progressive brain damage and usually die by 2 years of age . in the third category ,
called type 3 , liver and spleen enlargement is variable , and signs of brain involvement such as seizures gradually become apparent .
atypical bone pain , pathological fractures , avascular necrosis and extremely painful bone crises have a great impact on the quality of lives of many .
in addition to clinical suspicion , some morphologic , hematologic and biochemical indicators can help establish the diagnosis . in a study conducted
, the proportion of correct diagnosis ( and treatment ) of gaucher patients in germany is only between 10 to 20% .
the history of treatment of gaucher 's disease started with splenectomy and continued with bone marrow transplantation , before the recent introduction of safe and effective enzyme replacement therapy .
intravenous administration of the enzyme results in the breakdown of accumulated glycolipids and , subsequently , in reversal of the manifestations of the disease . in gaucher disease today , splenectomy alleviates hematologic abnormalities in patients with hypersplenism , but it does not correct the underlying disease process .
pollock et al . , showed that even after a successful open splenectomy procedure , morbidity was about 21% and mortality was 2% . the morbidity and mortality increase especially in patients with the spleen of extremely great size .
concept , the diagnosis of gaucher disease should be made with less invasive methods than surgery .
if the surgeon decided to perform splenectomy due to splenomegaly or splenic mass , the gaucher disease should be considered .
 context : splenectomy continues to find common therapeutic indications for hematologic disorders . 
 in addition , recently it is also performed in surgical clinics to assist diagnose of some illnesses . 
 gaucher 's disease , especially type i , is the most frequently encountered lysosomal storage disorder in man . 
 manifestations of it are highly variable . 
 the most frequently found symptoms include splenomegaly with anaemia and thrombocytopenia , mostly due to hypersplenism , hepatomegaly and bone disease.cases:four patients were reported in the present study . 
 three of them were easily diagnosed with gaucher 's disease via bone marrow cytology , and one with gaucher 's disease was detected by pathological examination following the splenectomy.conclusions:for the pouse of diagnosis of the gaucher 's disease , performing surgery is generally not necessary . however , for the cases of difficult to diagnose by classical methods , the corect diagnosis of gaucher 's disease can only be made by a special operation . 