_____________________________________________
A practical Approach to hepatosplenomegaly
(HSM )
_____________________________________________
♻️ كلنا بنشوف حالات جايه بشكوى معينه ولكن بالفحص او بالسونار بنكتشف وجود hepatosplenomegaly ،، فى الوقت ده ممكن نتلخبط وما نعرفش نفكر ازاى عشان نقدر نوصل للتشخيص
📝📝📝 عشان نفكر بشكل علمى مظبوط للوصول للتشخيص هنتبع ال Algorithm ده او خارطة الطريق دى ⤵️⤵️⤵️⤵️
ROAD MAP TO HSM#
📎 اولا بص على عين المريض لو لقيت jaundice هتتبع الخطوات الاتيه كالاتى 👇👇👇👇
A} IF THERE IS JAUNDICE:-
→Next step:
Search for Stigmata of Chronic liver disease{ CLD}.
📎 لو المريض عنده HSM + jaundice + علامات CLD
Stigmata for CLD
palmar erythema, spider naevi, leuconychia, Gynecomastia, ASCITIS,, LL edema.
مش لازم يكونوا كلهم موجودين ،،
وجود Ascitis ± LLedema بيدل على ان المريض
Decompensated
→ If present ,so➡ It is Liver cirrhosis.
Possibilties
It is either primary liver disease or Cardiac cirrhosis.
📎 وصولك لنقطة ان المريض عندة تليف كبدى مش تشخيص كافى لابد من معرفة السبب عشان العلاج
So next step ⬇⬇⬇⬇
Search for signs of cardiac disease>>>>Increased JVP & congested neck veins , tender hepatomegaly +/- basal crepitations on chest or signs of chronic lung disease like COPD --> cor pulmonale---> if positive➡ it is cardiac cirrhosis
Diagnostic workup
Basic :
LFT, RFT, CBC, Coagulation profile
Specific
Echo, CXR.
If no signs of cardiac disease>>
It is Liver cirrhosis.
Possibilities
*HCV & HBV.
* metabolic.
- NASH( central obesity +/- diabetes )
- Alcoholic ( ask about alcohol intake).
- Hemochromatosis ( bronzed skin, DM, cardiac affection, impotence).
- Wilson ( young < 40 years, neurological affections ( ataxia, tremors).
* Autoimmune:
- Autoimmune hepatitis ( more in young to middle aged females, other autoimmune diseases like thyroid)
- Primary biliary cirrhosis ( middle aged females)
- primary sclerosing cholangitis ( males , history of ulcerative colitis).
So, next diagnostic workup
*Basic for assessment of liver function
LFT, RFT, CBC, PT/INR
U/ S abdomen +/- Upper endoscopy.
*to detect the cause
Check viral markers
HCV Ab , HBS antigen.
If negative
⬇⬇⬇⬇
Do the following:
*Lipid profile, Fasting blood glucose, HbA1C ---> for NASH.
* ferritin & transferrin saturation for hemochromatosis.
* 24 hrs urine collection with estimation of copper in urine for Wilson.
* ANA, ASMA, anti-LKM, anti- SLA and globulins for AIH.
* AMA for PBC
* MRCP for PSC.
* if all of the above are inconclusive, do liver biopsy.
ملحوظه هااامه :
✅✅✅ The prescence of Ascitis & LL edema denotes that the patient has decompensated Liver Cirrhosis , so searching for the cause is not useful , because the definite ttt and the only hope as well ,is liver transplantation
🌟 اما لو مفيش علامات decompensation ،، لابد من البحث عن السبب ،، لان العلاج بيفرق طبعا فى حالات زى ال AIH و ال NASH وال Wilson وغيرهم
🔲🔲🔲🔲
📎طب لو HSM + jaundice
من غير علامات CLD
🌀If there is HSM+JAUNDICE
Without stigmata of CLD
So , ⬇⬇⬇⬇⬇⬇
Possibilities
Chronic hemolytic anemias:-
* Thalassemia major ( history of DX, history of blood transfusion, Mongoloid features).
* Hereditary Spherocytosis( history of recurrent hemolytic jaundice + positive family history )
So, diagnostic workup
Basic labs including
CBC, LFT( total & indirect bilirubin), LDH,
Retics, haptoglobin, urine analysis
U/S abdomen& pelvis.
To detect the cause:
HGB electrophoresis.
Osmotic fragility test.
--------------------------------------------------------------------------
*****************
📎 بعدين هتحس ال Lymph Nodes لولقيتها متضخمه !! ⤵️⤵️⤵️⤵️⤵️⤵️⤵️
B} if there is HSM + lymphadenopathy:
Possibilities
* Hodgkin's & Non Hodgkin's lymphoma.
* CLL ( above 50 years old, lymphocytic leucocytosis, absolute lymphocytic count > 5000).
* hairy cell leukemia( marked splenomegaly + pancytopenia)..
A practical Approach to hepatosplenomegaly
(HSM )
_____________________________________________
♻️ كلنا بنشوف حالات جايه بشكوى معينه ولكن بالفحص او بالسونار بنكتشف وجود hepatosplenomegaly ،، فى الوقت ده ممكن نتلخبط وما نعرفش نفكر ازاى عشان نقدر نوصل للتشخيص
📝📝📝 عشان نفكر بشكل علمى مظبوط للوصول للتشخيص هنتبع ال Algorithm ده او خارطة الطريق دى ⤵️⤵️⤵️⤵️
ROAD MAP TO HSM#
📎 اولا بص على عين المريض لو لقيت jaundice هتتبع الخطوات الاتيه كالاتى 👇👇👇👇
A} IF THERE IS JAUNDICE:-
→Next step:
Search for Stigmata of Chronic liver disease{ CLD}.
📎 لو المريض عنده HSM + jaundice + علامات CLD
Stigmata for CLD
palmar erythema, spider naevi, leuconychia, Gynecomastia, ASCITIS,, LL edema.
مش لازم يكونوا كلهم موجودين ،،
وجود Ascitis ± LLedema بيدل على ان المريض
Decompensated
→ If present ,so➡ It is Liver cirrhosis.
Possibilties
It is either primary liver disease or Cardiac cirrhosis.
📎 وصولك لنقطة ان المريض عندة تليف كبدى مش تشخيص كافى لابد من معرفة السبب عشان العلاج
So next step ⬇⬇⬇⬇
Search for signs of cardiac disease>>>>Increased JVP & congested neck veins , tender hepatomegaly +/- basal crepitations on chest or signs of chronic lung disease like COPD --> cor pulmonale---> if positive➡ it is cardiac cirrhosis
Diagnostic workup
Basic :
LFT, RFT, CBC, Coagulation profile
Specific
Echo, CXR.
If no signs of cardiac disease>>
It is Liver cirrhosis.
Possibilities
*HCV & HBV.
* metabolic.
- NASH( central obesity +/- diabetes )
- Alcoholic ( ask about alcohol intake).
- Hemochromatosis ( bronzed skin, DM, cardiac affection, impotence).
- Wilson ( young < 40 years, neurological affections ( ataxia, tremors).
* Autoimmune:
- Autoimmune hepatitis ( more in young to middle aged females, other autoimmune diseases like thyroid)
- Primary biliary cirrhosis ( middle aged females)
- primary sclerosing cholangitis ( males , history of ulcerative colitis).
So, next diagnostic workup
*Basic for assessment of liver function
LFT, RFT, CBC, PT/INR
U/ S abdomen +/- Upper endoscopy.
*to detect the cause
Check viral markers
HCV Ab , HBS antigen.
If negative
⬇⬇⬇⬇
Do the following:
*Lipid profile, Fasting blood glucose, HbA1C ---> for NASH.
* ferritin & transferrin saturation for hemochromatosis.
* 24 hrs urine collection with estimation of copper in urine for Wilson.
* ANA, ASMA, anti-LKM, anti- SLA and globulins for AIH.
* AMA for PBC
* MRCP for PSC.
* if all of the above are inconclusive, do liver biopsy.
ملحوظه هااامه :
✅✅✅ The prescence of Ascitis & LL edema denotes that the patient has decompensated Liver Cirrhosis , so searching for the cause is not useful , because the definite ttt and the only hope as well ,is liver transplantation
🌟 اما لو مفيش علامات decompensation ،، لابد من البحث عن السبب ،، لان العلاج بيفرق طبعا فى حالات زى ال AIH و ال NASH وال Wilson وغيرهم
🔲🔲🔲🔲
📎طب لو HSM + jaundice
من غير علامات CLD
🌀If there is HSM+JAUNDICE
Without stigmata of CLD
So , ⬇⬇⬇⬇⬇⬇
Possibilities
Chronic hemolytic anemias:-
* Thalassemia major ( history of DX, history of blood transfusion, Mongoloid features).
* Hereditary Spherocytosis( history of recurrent hemolytic jaundice + positive family history )
So, diagnostic workup
Basic labs including
CBC, LFT( total & indirect bilirubin), LDH,
Retics, haptoglobin, urine analysis
U/S abdomen& pelvis.
To detect the cause:
HGB electrophoresis.
Osmotic fragility test.
--------------------------------------------------------------------------
*****************
📎 بعدين هتحس ال Lymph Nodes لولقيتها متضخمه !! ⤵️⤵️⤵️⤵️⤵️⤵️⤵️
B} if there is HSM + lymphadenopathy:
Possibilities
* Hodgkin's & Non Hodgkin's lymphoma.
* CLL ( above 50 years old, lymphocytic leucocytosis, absolute lymphocytic count > 5000).
* hairy cell leukemia( marked splenomegaly + pancytopenia)..
Acute lymphoblastic leukemia( young patients( marked splenomegaly, pancytopenia or high leucocytic count with predominant lymphocytes in differential)
Diagnostic work up:
⬇⬇⬇⬇⬇
basic labs including
CBC with blood film( blasts in ALL)
ESR
LDH
Bone marrow aspirate /biopsy immunophenotyping .
Imaging in lymphoma( CT or PET CT).
--------------------------------------------------------------------------
*************
📎 طب لو لقيت صورة دم غير طبيعيه مع HSM بعد ما استبعدت الاسباب السابقه ⤵️⤵️⤵️⤵️⤵️
C} if there is Abnormalities in CBC with HSM:
1) Pancytopenia or bicytopenia:
Possibilities:
* ALL/AML( young, weight loss, fever, pallor, sore throat and marked splenomegaly).
* Hairy cell leukemia( mentioned before)
* Myelofibrosis ( old age, marked fatigue, marked splenomegaly).
* Hypersplenism due to portal hypertension ( mentioned before).
Diagnostic workup:
Basic labs including:
Blood film( ALL,AML)
ESR, Retics
JAK 2 mutation for Myelofibrosis.
Bond marrow aspirate +biopsy ( essential in all possibilties)
Immunophenotyping.
2) marked increase in one or more cell lines in CBC:
* high HgB/ high WBCs/high platelets
>>> Polycythemia vera
Next step
JAK2 mutation +/- bone marrow aspirate.
* if WBCs are high with predominant granulocytes + anemia
>>>>> CML
Next step
Philadelphia chromosome
Bone marrow aspirate.
* if high platelets particularly if > 1,000,000 +/- anemia
>>>>> Essential thrombocytosis
Next step
JAK2 mutation + bone marrow aspirate.
--------------------------------------------------------------------------
**************************
📎 طب لو كان المريض معاه ارتفاع فى درجة الحراره من الاول مع ال HSM ⤵️⤵️⤵️⤵️⤵️⤵️⤵️
D} If there is FEVER:
It is suggestive of Infectious causes then think also about acute leukemia/Lymphomas
Possibilities:
* Acute Viral hepatitis ( tender hepatomegaly mainly):-
→ hepatotropic ( A, B, C, E)
→ Non hepatotropic { CMV , EBV or infectious mononucleosis}.
Diagnostic work up:
⬇⬇⬇⬇⬇
basic labs including
CBC with blood film( blasts in ALL)
ESR
LDH
Bone marrow aspirate /biopsy immunophenotyping .
Imaging in lymphoma( CT or PET CT).
--------------------------------------------------------------------------
*************
📎 طب لو لقيت صورة دم غير طبيعيه مع HSM بعد ما استبعدت الاسباب السابقه ⤵️⤵️⤵️⤵️⤵️
C} if there is Abnormalities in CBC with HSM:
1) Pancytopenia or bicytopenia:
Possibilities:
* ALL/AML( young, weight loss, fever, pallor, sore throat and marked splenomegaly).
* Hairy cell leukemia( mentioned before)
* Myelofibrosis ( old age, marked fatigue, marked splenomegaly).
* Hypersplenism due to portal hypertension ( mentioned before).
Diagnostic workup:
Basic labs including:
Blood film( ALL,AML)
ESR, Retics
JAK 2 mutation for Myelofibrosis.
Bond marrow aspirate +biopsy ( essential in all possibilties)
Immunophenotyping.
2) marked increase in one or more cell lines in CBC:
* high HgB/ high WBCs/high platelets
>>> Polycythemia vera
Next step
JAK2 mutation +/- bone marrow aspirate.
* if WBCs are high with predominant granulocytes + anemia
>>>>> CML
Next step
Philadelphia chromosome
Bone marrow aspirate.
* if high platelets particularly if > 1,000,000 +/- anemia
>>>>> Essential thrombocytosis
Next step
JAK2 mutation + bone marrow aspirate.
--------------------------------------------------------------------------
**************************
📎 طب لو كان المريض معاه ارتفاع فى درجة الحراره من الاول مع ال HSM ⤵️⤵️⤵️⤵️⤵️⤵️⤵️
D} If there is FEVER:
It is suggestive of Infectious causes then think also about acute leukemia/Lymphomas
Possibilities:
* Acute Viral hepatitis ( tender hepatomegaly mainly):-
→ hepatotropic ( A, B, C, E)
→ Non hepatotropic { CMV , EBV or infectious mononucleosis}.
* Bacterial( just palpable spleen)
Typhoid fever.
Brucellosis.
Leptospirosis.
Infective endocarditis .
* Protozoal :
Malaria.( huge spleen)
* ALL & AML( often moderate to huge splenomegaly)
* lymphoma( often palpable lymph nodes).
★ Next step, look for jaundice:-
If there is jaundice + Fever+ HSM---->
Possibilities:
*Acute viral hepatitis ( A, B, C, E, EBV, CMV). Fever followed by jaundice.
* Leptospirosis( combined liver+ kidney injury).
So, Diagnostic work up :
→LFT, RFT, CBC, CRP, Coagulation profile
→To detect the definite cause:
HAV IgM, HBc IgM, HCV RNA, HEV IgM, EBV IgM, CMV IgM
Leptospira antibody or culture for it.
Second step >>>>> If there is no jaundice so, Possibilities:
⬇⬇⬇⬇⬇⬇
*CMV infection.
*Infectious mononucleosis.
*Typhoid fever.
*Brucellosis.
*Infective endocarditis
*Malaria ( only in endemic areas or history of travel to endemic areas like Africa).
*ALL/ AML.
*Lymphomas.
So , Diagnostic workup
General labs:
>>CBC with blood film( for blasts in acute leukemias , also helpful in specific types of lymphomas).
ESR, CRP, RFT, Coagulation profile
To detect the causes:
⬇⬇⬇⬇⬇
*CMV IgM, Monospot test for infectious mononucleosis.
*Widal test & blood culture for typhoid.
*Brucella antibody titre for brucellosis.
*Blood culture & ECHO for infective endocarditis.
* bone marrow aspirate & immunophenotyping for ALL& AML
* Lymph node biopsy , bone marrow biopsy , immunophenotyping for lymphomas.
--------------------------------------------------------------------------
********************
📎 بعد كده لو مفيش سبب من اللى فاتوا لازم تستبعد ال Sarcoidosis ⤵️⤵️⤵️
E) if there is lung affection( interstitial lung disease) + skin lesion( erythema nodosum/lupus pernio+/-cranial nerve palsies+/- cardiac affection ( restrictive pattern..
>>>>>> Sarcoidosis
Next step
Serum ACE
Basic labs & ESR
CT chest with transbronchial biopsy ( gold standard of DX in cases with lung affection).
--------------------------------------------------------------------------
***********
📎 وكمان ال Amyloidosis ⤵️⤵️⤵️⤵️⤵️⤵️
F} if there is associated Large tongue+ cardiac affection+ renal affection + neuropathy+ periorbital purpura
>>>> Amyloidosis.
Next step
Basic labs including
Total protein
Protein electrophoresis +/- immunofixation
Lamda/kappa light chains in urine.
Subcutaneous pad of fat and biopsy with Congo red stain.
--------------------------------------------------------------------------
************
📎 طب لو لقيت تضخم كبير فى الطحال
Spleen crossing the umbilicus
الاحتمالات كالاتى 👇
G ) if there is Huge splenomegaly
Possibilities:
⬇⬇⬇⬇⬇⬇⬇
* Malaria( endemic areas/ high fever/)
Next step : thin& thick blood film for malaria
Typhoid fever.
Brucellosis.
Leptospirosis.
Infective endocarditis .
* Protozoal :
Malaria.( huge spleen)
* ALL & AML( often moderate to huge splenomegaly)
* lymphoma( often palpable lymph nodes).
★ Next step, look for jaundice:-
If there is jaundice + Fever+ HSM---->
Possibilities:
*Acute viral hepatitis ( A, B, C, E, EBV, CMV). Fever followed by jaundice.
* Leptospirosis( combined liver+ kidney injury).
So, Diagnostic work up :
→LFT, RFT, CBC, CRP, Coagulation profile
→To detect the definite cause:
HAV IgM, HBc IgM, HCV RNA, HEV IgM, EBV IgM, CMV IgM
Leptospira antibody or culture for it.
Second step >>>>> If there is no jaundice so, Possibilities:
⬇⬇⬇⬇⬇⬇
*CMV infection.
*Infectious mononucleosis.
*Typhoid fever.
*Brucellosis.
*Infective endocarditis
*Malaria ( only in endemic areas or history of travel to endemic areas like Africa).
*ALL/ AML.
*Lymphomas.
So , Diagnostic workup
General labs:
>>CBC with blood film( for blasts in acute leukemias , also helpful in specific types of lymphomas).
ESR, CRP, RFT, Coagulation profile
To detect the causes:
⬇⬇⬇⬇⬇
*CMV IgM, Monospot test for infectious mononucleosis.
*Widal test & blood culture for typhoid.
*Brucella antibody titre for brucellosis.
*Blood culture & ECHO for infective endocarditis.
* bone marrow aspirate & immunophenotyping for ALL& AML
* Lymph node biopsy , bone marrow biopsy , immunophenotyping for lymphomas.
--------------------------------------------------------------------------
********************
📎 بعد كده لو مفيش سبب من اللى فاتوا لازم تستبعد ال Sarcoidosis ⤵️⤵️⤵️
E) if there is lung affection( interstitial lung disease) + skin lesion( erythema nodosum/lupus pernio+/-cranial nerve palsies+/- cardiac affection ( restrictive pattern..
>>>>>> Sarcoidosis
Next step
Serum ACE
Basic labs & ESR
CT chest with transbronchial biopsy ( gold standard of DX in cases with lung affection).
--------------------------------------------------------------------------
***********
📎 وكمان ال Amyloidosis ⤵️⤵️⤵️⤵️⤵️⤵️
F} if there is associated Large tongue+ cardiac affection+ renal affection + neuropathy+ periorbital purpura
>>>> Amyloidosis.
Next step
Basic labs including
Total protein
Protein electrophoresis +/- immunofixation
Lamda/kappa light chains in urine.
Subcutaneous pad of fat and biopsy with Congo red stain.
--------------------------------------------------------------------------
************
📎 طب لو لقيت تضخم كبير فى الطحال
Spleen crossing the umbilicus
الاحتمالات كالاتى 👇
G ) if there is Huge splenomegaly
Possibilities:
⬇⬇⬇⬇⬇⬇⬇
* Malaria( endemic areas/ high fever/)
Next step : thin& thick blood film for malaria
* Visceral leishmaniasis( endemic areas/ pancytopenia
Next step : leishmanin test.
* ALL/ AML >>> blood film+ bone marrow aspirate.
* PRV>>>> JAK2 mutation+/- bone marrow aspirate)
* CML>>>>> Philadelphia chromosome+ bone marrow aspirate.
* Myelofibrosis >>>> blood film+ bone marrow biopsy + JAK2 mutation.
* Bilharziasis >>> rectal snip+ anti- bilharzial antibody + U/S abdomen.
* Splenic marginal zone lymphoma
>>>> immunophenotyping.
+ splenectomy with histopatholog
Next step : leishmanin test.
* ALL/ AML >>> blood film+ bone marrow aspirate.
* PRV>>>> JAK2 mutation+/- bone marrow aspirate)
* CML>>>>> Philadelphia chromosome+ bone marrow aspirate.
* Myelofibrosis >>>> blood film+ bone marrow biopsy + JAK2 mutation.
* Bilharziasis >>> rectal snip+ anti- bilharzial antibody + U/S abdomen.
* Splenic marginal zone lymphoma
>>>> immunophenotyping.
+ splenectomy with histopatholog
جاري تحميل الاقتراحات...