Emirates Society of Internal medicine · Transcript · · Updated
02 Hasan Aal Yaseen Approach to Thrombocytopenia anemia Pancytopenia – Transcript
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- Pancytopenia is defined as a reduction of all three blood elements in the peripheral blood.
- Anemia can be categorized as relative due to an increased plasma volume or absolute due to a reduction of the red cell mass.
- True thrombocytopenia can be investigated using a peripheral blood film for schistocytes, blast cells, or dysplastic features.
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so I was asked to uh talk about approach to panasonia and anemia in 15 minutes I'll do my best but I don't I I cannot guarantee that okay I'll start with pansytopenia as the name suggests pancytopenia is a reduction of all the three blood elements in the peripheral blood it is a very common reason to refer to a hematologist so out of 10
cases of pancytopenia nine of them are being referred to hematologists and we have to realize that pancytopenia is not a disease by itself it is a result of an underlying pathology either in the bone marrow or the peripheral blood and this concept applies for anemia as well it is not a disease it is a result of underlying pathology however
thrombocytopenia does represent clinical and and pathophysiological entities so how do patients present they either present accurately or chronically and this will help us in the diagnosis these patients may be actually discovered incidentally though May there are maybe asymptomatic however they can
be very ill critically ill in the ICU as well and the main symptoms or clinical presentation of pancytopenia depends on the elements so they will present with symptoms of anemia recurrent of infections due to leukopenia plus minus bleeding due to thrombocytopenia which is not necessarily the case now how do you investigate a patient with pancytopenia there are some basic
required investigations that should be done with each case of pancytopenia and from there you go to your specific workup so of course a full blood count and then a peripheral blood smear rat account possibly LDH these four are mandatory for any case of pancytopenia in order to know in which direction you you are going to and then of course you can
include liver function test renal functions this should be included initially because they are basic tests coagulation ptptcrp 13 and from that you will you know in which direction you are regarding the etiology so you can then order virology screen for the famous parvovirus HIV hepatitis C Hepatitis B and of course our new guest covet you can you can request of course
according to the presentation and the result uh labs for uh collagen vascular disease flow cytometry for pnh if you suspect a pnh chronic infections like TV leishmania malaria and if you are suspecting congenital ecclesias then you can order NGS and of course bone marrow aspiration and biopsy
is one of the main Corner uh tests that should be done for pancytopenia accordingly and if patients have palpable lymph nodes you have to take one lymph node for histology now what are the main categories of etiology of pancytopenia we can categorize them and to impair the production and these represent a central cause of pancytopenia and peripheral destruction
where elements of the blood IB are being distracted peripherally or sequestrated in the spleen however keep in mind that pancytopenia does not necessarily default in in one of these categories we can have multiple causes of each case of pancytopenia examples for impaired production of course of course Central causes in the bone marrow like aplastic anemia whether congenital or acquired we
can have infiltration of the bone marrow like any malignancy infiltrating the vomoral infections or a primary bone marrow disorder like a plastic anemia multiple myeloma here is a leukemia chronic leukemias myelofibrosis is a very famous cause of pancytopenia granulomatous disorders metabolic disorders and we have nutritional deficiencies vitamin B12
folic and copper and of course we shouldn't forget MDS myelogenous plastic syndrome now what are the peripheral causes of pancytopenia we have mainly immune so we can have immune mainly immune disytopenia or immune pancytopenia so these the elements will be distracted by an immune process peripherally and we can have the elements of the blood sequestrated in the case of high personalism and
spinach sequestration examples of of a combination of causes or etiology can include a famous example of pnh so npnh you have bone marrow hypoplasia and a chronic Coombs negative intravascular hemolysis and the other examples that are listed so keep in mind that in each case or or in some of the cases of pancytopenia you can have multiple causes for pancytopenia even in
the General Medical cause like chronic liver disease you can have high parasolism sepsis folate deficiency and accordingly probably alcoholic being alcoholic liver disease as a cause of chronicity of the liver disease now I I just choose the copper deficiency why because it is is slightly and and thought about you you don't usually think about copper deficiency it mainly
causes neutropenia and the thrombocytopenia however it can cause pancytopenia as well and in the bone marrow you will see vacuoes as you can as you can see here both and the myeloid and in the erythroid lineage and the erythroid these vacuoes are suggestive of copper deficiency as a cause of dicyclopedia or pancytopenia and this
graph just tells you that the etiology is actually of pancytopenia is dependent on many other factors and we will talk about this in the coming slides so etiologists differs according to the geography according to the socioeconomic status so this this retrospective study was conducted in Pakistan and as you can see here a
nutritional causes of pancytopenia ranked the first while AML aplasia and erythroid hyperplasia came later I'm sure that if there is a retrospective study in USA or Europe probably the sequence of of etiological causes will will differ now there are hints that you have to keep in mind and when you approach a
case of pancytopenia we have epidemic epidemiological hints we have probably clinical hands so epidemiological hence as I said that pancytopenia uh the causes of pancytopenia differs according to the socioeconomic status according to geography so in areas where you have limited resources you should think about a mild absorption about infections
however thanks to bariatric surgery still you should think about uh for about nutritional deficiency because of that uh HIV alcohol as causes of pancytopenia are related to some social behavioral patterns and age is a very important factor when you when you approach a case of pancytopenia in children you have to
bear in mind congenital causes of bone marrow hypoplasia while in adults you have to think about acquired causes however keep in mind that that some causes of bone marrow aplasia appearing in adults or hlh can be due to genetic inborn errors which have low penetrance and they only show later and Elder and adulthood and they sometimes need a
trigger factor and external trigger Factor so those were the epidemiological hints what about the clinical hints that might help us to approach cases of pancytopenia if pancytopenia is acute so we have to think about sepsis or educing critic drug reactions all the other causes are mainly or probably will lead to sub-acute or chronic
pancytopenia now the severity of pancytopenia you rarely have severe parasite opinion in cases of megaloblastic anemia or in hypersplanism especially due to the versurosis while for with bone marrow aplasia aplastic anemia the pancytopenia is severe with erratic count of less than 500 platelets of less than 20 having said that just
remember that in megaloblastic anemia specifically they may present with severe anemia reaching even two we have seen cases of megaloblastic anemia patients attending the emergency with the hemoglobin of two how the platelets and the white blood cells are not usually so low so so the platelets will be in the area of 40-7 to 70. now macrocytosis with pancarttopenia you
should think about aplastic anemia mdslastic anemia probably multiple myeloma some medications leading to paracetopenia like hydroxyurea and possibly immune pancytopenia of the splenomegaly this is a very common clinical and theoretical question exam exam question I mean so when you see a patient with pancytopenia having a
palpable spleen you should think about chronic liver disease with hypersalinism infections viral malaria leishmania TB hematological malignancy hairy cell leukemia lymphomas and the mpns myeloproliferative neoplasm mainly myelofibrosis like in thalassemias chronic inflammatory processes like collagen vascular disease filthy syndrome
Rheumatic and rheumatoid arthritis storage diseases like gaucher disease or hemorphicocytic syndrome now if the labs tell you that this there's DIC with pancytopenia keep in mind acute bromyocytic leukemia as a cause but of course you have to consider sepsis if you have hyperferitonemia with pants with pancytopenia of course you
have to consider hlh now this is a a graph or an algorithm which is suggested by this group of Investigation investigators who published in review and blood reviews and this is basically a summary of what we have just said uh the aim of uh having this slide is to
pass a message that sometimes at the end you may not reach a reason for pancytopenia and this entity is labeled as idiopathic cytopenia of undetermined significance so just like gammopathy of undetermined significance or or other clinical entities of undetermined significance we have idiopathic cytopenia of undetermined significance which is
thought mainly to be due to immune reasons now just this is just to show you how can peripheral pancytopenia be associated with a hypoplastic bone marrow like in a plastic anemia or hypoplastic MDS while you can have empty blood and full bone marrow so empty blood
pancytopenia and full bone marrow like in megaloblastic anyway so bone marrow is hyper cellular while the peripheral blood is passed out to panic this is seen in megaloblastic anemia in MDS in hypertensylenism and in cases of infiltration of the bone model okay now what about anemia so this was about approach to pancytopenia what about approach to anemia keep in mind
that anemia can be a relative or absolute so relative anemia is due to an increase in the plasma volume rather than a reduction of the Red Cell Mass an increase in the plasma for volume can can happen with microglopidinemia and with the pregnancy sometimes with the astronauts while reduction of the Red Cell Mass