It's a common, but poorly understood, gynecological condition that affects women of all age groups. It is defined as the presence of ectopic nests of endometrial glands and stroma within the myometrium, surrounded by reactive smooth muscle hyperplasia. Adenomyosis is a common cause of dysmenorrhea, menorrhagia, and chronic pelvic pain, but is often underdiagnosed.

It's a thrombotic microangiopathy (TMA) caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13. It is characterized by arteriolar platelet-rich thrombi that cause organ ischemia and produce neurologic abnormalities, kidney dysfunction, thrombocytopenia, and microangiopathic hemolytic anemia (MAHA).

Of all TTP, >90 percent is immune, caused by autoantibody-mediated inhibition of ADAMTS13 activity or clearance of ADAMTS13 protein.

MAHA & Thrombocytopenia are the hallmarks of TTP and should warrant suspicion of TTP in a patient with no alternative explanation.

Microangiopathic hemolytic anemia (MAHA) results from mechanical shearing (fragmentation) of RBCs as they pass through platelet-rich microthrombi in the microvasculature; it is documented by the finding of prominent schistocytes, including helmet cells and triangular cells, on the peripheral blood smear.

More on bleeding

Platelet/vessel wall defects cause spontaneous purpura in the skin and mucous membranes or immediately after trauma. Coagulation defects cause haematomas and haemarthroses usually with a time delay after trauma. A positive family history or early onset of bleeding suggests haemophilia.

The distribution of bruising may suggest the diagnosis:
• Thrombocytopenic purpura is most evident over the ankles and pressure areas. Retinal haemorrhages tend to occur if there is a combination of severe thrombocytopenia and anaemia.
• Senile purpura and steroid-induced bruising occur mainly on the forearms and backs of the hands.
• Henoch–Schönlein purpura typically occurs over the extensor aspects of the limbs and buttocks.
• Scurvy causes bleeding from the gums and around the hair follicles.

Schistocytes

In contrast with typical disseminated intravascular coagulation (DIC) seen with other causes of sepsis and occasionally with advanced cancer, coagulation factors are not consumed in HUS or TTP and the coagulation screen, fibrinogen level, and assays for fibrin degradation products such as "D-Dimers", are generally normal despite the low platelet count (thrombocytopenia).

It's basically the result of an infection with a bacteria that produces the shiga toxin (sometimes called Verotoxin). In the developing countries, it's usually caused by shigella spp., while in developed countries, enterohemorrhagic strains of E. coli usually are the culprits (the most famous being O157:H7).

The toxin binds preferentially to renal endothelium and causes AKI and renal microangiopathy (hence, it causes uremia). Thereafter, it disrupts the ADAMTS13 protein and causes TTP.

The signs and symptoms are usually:
• Bloody diarrhea
• AKI (and hypertension)
• signs and symptoms of TTP, most notably bleeding problems and neurological symptoms.

طبعًا أكو حالات HUS سببها يكون S. pneumoniae مثلًا. هاي الحالات تكون خطرة وإحتمالية الوفاة بيها توصل 50%

ها صدك
الـ TTP ممكن يكون الـ initial presentation مالتها أثناء الحمل¹ وهنا الله يساعد الطبيب المسؤول عن علاج المريضة، لأن يرادله إستخارة ودعاء الوالدة يلا يميزها عن الـ preeclampsia and HELLP syndrome


¹ أصلًا نسبة الـ von Willebrand factor تزيد ونسبة الـ ADAMTS13 تقل أثناء الحمل، والحدثين ذولة ثنينهم يزيدون إحتمالية الإصابة بالـ TTP

Diabetic nephropathy (DM-N) causes thickening of glomerular basement membrane (GBM) along with mesangial proliferation & matrix expansion.

Therefore, DM-N usually causes nodular, but also diffuse glomerulosclerosis.